RESTRICTIVE LUNG DISEASES

INTRODUCTION
The Restrictive lung diseases are a heterogeneous set of pulmonary disorders defined by
restrictive patterns. These disorders are characterized by a reduced distensibility of the lungs,
compromising lung expansion, and, in turn, reduced lung volumes, particularly with reduced
total lung capacity (TLC) 1.
Restrictive lung diseases may also result from limitations in neuromuscular function and chest
wall movements (extrinsic causes). These may be due to neuromuscular diseases, pleural
disorders, obesity, costo sternal or costovertebral fusion, a fusion or deviation of the thoracic
vertebra, and other etiologies that result in a physical impediment to inspiration. Regardless of
the intrinsic or extrinsic mechanisms, these diseases are featured by impaired ventilatory
function and respiratory failure.
The prevalence and incidence of ILDs (Interstitial lung diseases) a population-based registry
of patients with ILDs (Interstitial lung diseases) was established in Bernalillo County, New
Mexico in October 1988. In addition, the prevalence of preclinical or undiagnosed cases was
identified by screening lung specimens from 510 autopsy cases. The overall prevalence of
restrictive diseases is difficult to estimate precisely because the groups involve multiple
pathological conditions, each of which can occur in numerous clinical stages. It has been
determined, however, that up to 3-6 cases per 100,000 persons suffer from intrinsic lung
diseases in the United States 2.
The pathophysiology effects of diffuse parenchymal disorders reduce all lung volumes by the
excessive elastic recoil of the lungs, relative to the outward recoil forces of the chest wall.
Expiratory airflow is reduced in proportion to lung volume. Arterial hypoxemia in disorders of
pulmonary parenchyma is primarily caused by ventilation-perfusion mismatching, with further
contribution from an intrapulmonary shunt. Decreased diffusion of oxygen rarely contributes
to hypoxemia because sufficient time still exists for full equilibration of oxygen or carbon
dioxide.
It can be mainly classified into Intrinsic and extrinsic restrictive lung disease the intrinsic
restrictive lung disease is idiopathic pulmonary fibrosis. Some other health issues that may be
involved in intrinsic restrictive lung disease include: Sarcoidosis, connective tissue diseases,
such as scleroderma, drug-induced lung disease drug-induced lung disease3.
Extrinsic restrictive lung diseases involve the chest wall, pleura, and respiratory muscles. The
pleura is a membrane that covers the inside surface of the rib cage and spreads over the lungs.
Diseases of these structures result in lung restriction, impaired function, and respiratory failure.
health issues that can be involved in extrinsic restrictive lung disease include: a buildup of fluid
between the layers of tissue surrounding the lungs, known as a pleural Effusion scoliosis, or
twisting of the spine, Obesity myasthenia gravis, or intermittent muscle weakness, rib damage,
especially fractures, diaphragm paralysis, kyphosis, or hunching of the upper back.

1
The acronym "PAINT" can be used to memorize the causes of restrictive lung disease into
pleural, alveolar, interstitial, neuromuscular, and thoracic cage abnormalities. Nevertheless, a
more accurate division takes into account the pathogeneic mechanism. Restrictive lung
diseases can be caused due to interstitial lung disease. This is an umbrella term for a large group
of diseases that cause scarring of lungs. The scarring leads to stiffness that makes it difficult to
breath and get oxygen to the blood stream .

People with different restrictive lung diseases have some symptoms, including: shortness of
breath, especially with exertion, long-term cough, gasping for breath fatigue, which can be
extreme, Depression, Anxiety.

Risk factors can be caused by exposure to hazardous materials. Ex; occupational exposures
such as asbestos or silica. Can be caused due to cigarette smoking. Caused by breathing in bird
or animal droppings. Can be caused due to Radiation treatments and certain types of
medications.

The diagnosis in radiographic, abnormality is a reticular pattern. Nodular, reticulonodular, or

mixed patterns, such as alveolar filling (i.e., ground-glass appearance), and increased
interstitial markings are not unusual; however, these are not predictive of a specific
pathological picture. High-resolution CT scanning can be helpful in such cases by providing
an accurate assessment and is recommended before lung biopsy.

Medical treatment for restrictive lung disease is normally limited to supportive care since both
the intrinsic and extrinsic causes can have irreversible effects on lung compliance. The
supportive therapies focus on maximizing pulmonary function and preserving activity
tolerance through oxygen therapy, bronchodilators, inhaled beta-adrenergic agonists, and
diuretics. Because there is no effective treatment for restrictive lung disease, prevention is key.

In surgical management Lung transplants offer a chance of a healthier, longer life, but they are
also highly risky. After a lung transplant, a person can develop life-threatening complications,
such as organ rejection. After a lung transplant, a recipient must take drugs that suppress the
immune system. However, these drugs make transplant recipients more vulnerable to many
infections. As a result, most transplant recipients must be closely monitored in hospital for
several weeks.

Physiotherapists play an important role in prescribing, supervising, in exercise. It is the use of

guideline-based exercise prescription methodology (using FITT methodology - Frequency,
Intensity, Time, and Type). Exercise prescription should include supervised aerobic exercise
and progressive resistance exercise. Exercise should be individually prescribed according to
the initial assessment and goals should be identified and agreed. Participants' exertion should
be regularly monitored, paying particular attention to chest pain, discomfort or breathlessness.
the BORG scale can be used .There are various physiotherapy treatments incorporated within
chest physiotherapy, breath conditioning techniques, such as pursed-lip breathing, slow, deep
breathing, or diaphragmatic breathing upper and lower limb strengthening and conditioning
exercises respiratory muscle strengthening exercises ,level walking, relaxation or visualized
meditation Eating a balanced, nutritious diet, quitting smoking, avoiding environments with
toxins, irritants, and allergens that may worsen symptoms 4

2
 EPIDEMIOLOGY

The prevalence and incidence of ILDs a population-based registry of patients with ILDs was
established in Bernalillo County, New Mexico in October 1988 In addition, the prevalence of
preclinical or undiagnosed cases was identified by screening lung specimens from 510 autopsy
cases. A total of 2,936 referrals were screened; 8.8% were prevalent cases and 6.9% were
incident cases. Overall, the prevalence of ILDs was 20% higher in males (80.9 per 100,000)
than in females (67.2 per 100,000). Similarly the overall incidence of ILDs was slightly more
common in males (31.5 per 100,000/year) than females (26.1 per 100,000/year)5.
The estimated prevalence of preclinical or undiagnosed ILDs among all deaths was 1.8%. The
most common incident diagnosed among both sexes were pulmonary fibrosis and idiopathic
pulmonary fibrosis, together accounting for 46.2% of all ILD diagnoses in males and 44.2% in
females. We conclude that the occurrence of ILDs in the general population may be more
common than previous estimates.
National and global population studies demonstrated that there are certain classes of individuals
reported to be at a higher risk for having a restrictive pattern on spirometry. The populations
with higher associations to restrictive lung patterns include:
Elderly - The prevalence of restrictive conditions increases from 2.7 cases per 100,000 persons
in individuals aged 35-44 years to over 175 cases per 100,000 in those older than 75 years.
Nevertheless, sarcoidosis, collagen-vascular-associated diseases, and pulmonary Langerhans
cell histiocytosis occur in patients aged 20-40 years.
African Americans- Compared to Whites (10.9 cases per 100,000), the prevalence in this group
is 35.5 cases per 100,000 person.
Females- Sarcoidosis is slightly more common in women. Moreover, the female gender has
also been reported an increased risk for a restrictive pattern. Again, IPF is more common in
men than in women.
Obese individuals- Restrictive patterns are related to an elevated body mass index (BMI) with
a decrease in lung volumes attributed to an increased amount of central obesity.
Smokers- For instance, IPF individuals are usually current or past smokers.
Those that have specific occupational and environmental exposures are also at increased risk.
Frequently encountering substances at workplaces such as asbestos, coal dust, and several other
hazardous specks of dust can cause tissue alterations in the lungs leading to inflammation,
tissue scarring, and a restrictive pattern on spirometry. Restrictive lung diseases are a rare
occurrence during pregnancy, but if it is present, it is likely due to neuromuscular disease or
kyphoscoliosis.An interesting finding concerns not only the epidemiology of the established
pathology but functional subclinical alterations as well as the temporal evolution. In an
epidemiological study, Kurth et al. showed that restrictive patterns on spirometry were found
to fall from 7.2% to 5.4% from 1988-1994 and from 2007-2010, which may be related to
increased occupational safety precautions and falling rates of cigarette smoking 6

3
 DEFINITIONS

Restrictive lung diseases may be caused by the destruction of distal lung parenchyma due to
infiltrates from inflammation, toxins, and mechanisms yet to be elucidated (intrinsic
conditions) as well as extra parenchymal conditions (extrinsic causes). Within the former
group, there are diseases characterized by inflammatory changes involving the alveolar
interstitium with possible involvement of the peripheral bronchial structures. The conditions
leading to this destruction are encompassed among the interstitial lung diseases (ILDs).
- G Makker H.
Restrictive lung diseases are characterized by reduced lung volumes, either because of an
alteration in lung parenchyma or because of a disease of the pleura, chest wall, or
neuromuscular apparatus.
- Brandenberger C Agrawal

Restrictive lung disease (RLD) is highly prevalent and frequently disabling and is caused by a
myriad of both pulmonary and extrapulmonary conditions. It is defined as a reduction of total
lung capacity (TLC) below the 5th percentile of the predicted value, with a preserved one
second forced expiratory volume to forced vital capacity ratio (FEV1/FVC).
-Nina saxena

4
 ANATOMY
The Lungs occupying major portions of the thoracic cavity, leave little space for the heart,
which excavates more of the left lung. The two lungs hold heart thight between them, providing
it protection it rightly deserves. There are ten bronchopulmonary segments in each lung.
Lungs are a pair of respiratory organs situated in the thoracic cavity. Each lungs invaginates
the corresponding pleural cavity. Right and left lungs are separated by mediastinum
Lungs are spongy in texture. In the young, the lungs are brown or grey in colour, gradually it
becomes mottled black because of the deposition of inhaled carbon particles. The right lung
weighs about 700grams, it is about 50 to 100 grams heavier than left lung.

FEATURES
Each lung is conical in shape it has
-An apex
-A base resting on diaphragm
-Three borders i.e., anterior, posterior, inferior
-Two surfaces i.e. costal and medial surface is divided into vertebral and mediastinal
Parts.

Fig.1. Lungs

5
PLEURA
Each lung is invested by and enclosed in a serous pleural sac that consists of two continuous
membranes.
The visceral or pulmonary pleura invest the lungs, the parietal pleura line the pulmonary
cavities and adhere to the thoracic wall, mediastinum and diaphragm.
The parietal pleura consist of four parts: coastal pleura which lines the internal surface of the
thoracic wall, mediastinal pleura which lines the lateral aspect of the mediastinum,
diaphragmatic pleura which lines the superior surface of the diaphragm on each side of the
mediastinum, cervical pleura extends through the superior thoracic aperture into the root of the
neck, forming a cup-shaped dome over the apex of the lung.

Fig.2. pleura

PLEURAL CAVITY
The pleural cavity is the potential space between the visceral and parietal layers of the pleural
and it contains a capillary layer of serous pleural fluid which lubricates the pleural surfaces and
allows the layers to slide smoothly over each other during respiration. Surface tension created
by the pleural cavity provides the cohesion that keeps the lung surface in contact with the
thoracic wall.

6
 FISSURES AND LOBES OF THE LUNGS
The right lung is divided into 3 lobes (upper, middle, lower) by two fissures (oblique and
horizontal) the left lung is divided into 2 lobes by the oblique fissure
The oblique fissure cuts into the whole thickness of the lung, except at the hilum. It passes
obliquely downwards and forwards, crossing the posterior border about 6cm below the apex
and the inferior border about 5cm from the median plane. Due to the oblique plane of the
fissure, the lower lobe is more anterior.
In right lung, the horizontal fissure passes from the anterior border up to the oblique fissure
and separates a wedge-shaped middle lobe from upper lobe. The fissure runs horizontally at
the level of the fourth costal cartilage and meets the oblique fissure in midaxillary line.
The tounge-shaped projection of the left lung below the cardiac notch is called lingual. It
corresponds to middle lobe of the right lung.
The presence of the oblique fissure of each lung allows a more uniform expansion of whole
lung.

Fig.3.Fissures and lobes of lungs

TRACHEOBRONCHIAL TREE
All the respiratory passages from the trachea to the respiratory bronchioles are called the
tracheobronchial tree. The trachea divides at the sternal angle into right and left primary
bronchus which goes into the right and left lungs. Each bronchus enters the lung at a notch
called the hilum. Blood vessels and nerves also connect with the lungs here and together with
the bronchus forms a region called the root of the lungs.

7
The right main bronchus is larger in diameter and more vertical making it directly in line with
the trachea than the left main bronchus. Thus swallowed objects that accidentally enter the
lower respiratory tract are most likely to become lodged in the right main bronchus.

Fig.4.Tracheobronchial tree

BRONCHOPULMONARY SEGMENTS
Functionally, the lung is divided into a series of bronchopulmonary segments. The
bronchopulmonary segments are the largest subdivision of a lobe. They are separated from
adjacent segments by connective tissue septa and are also surgically resectable. They are 10
bronchopulmonary segments in the left lung and 8-10 in the left lung.
The bronchi further divides, finally giving rise to the bronchioles which are less than 1mm in
diameter. Each bronchioles divides into 50 to 80 terminal bronchioles, the final branches of
respiratory bronchioles. The functional unit of the lungs which is the acinus includes the
respiratory bronchioles, alveolar ducts, and sacs and the alveolar. Approximately 16
generations of branching occur from the trachea to the terminal bronchioles. As the air
passageways of the lungs become smaller, the structure of their walls changes.

8
 Fig.5.Bronchopulmonary segments

ARTERIAL SUPPLY
The bronchial arteries supply nutrition to the bronchial tree and pulmonary tissue. These are
small arteries that vary in number, size and origin, but usually they are as follows
On the right side, there is one bronchial artery which arises from the third right posterior
intercostal artery.
On the left side, there are two bronchial arteries, both of which arises from the descending
thoracic aorta, the upper opposite fifth thoracic vertebra and the lower just below the left
bronchus
Deoxygenated blood is brought to the lungs by two pulmonary arteries and oxygenated blood
is returned to heart by four pulmonary veins
There are precapillary anastomoses between bronchial and pulmonary arteries these
connections enlarge when one of them is obstructed in any disease.

9
 Fig.6.Arterial supply of lungs

NERVE SUPPLY
-Parasympathetic nerves are derived from vagus nerve. These fibres are:
A. Motor to the bronchial muscles and on stimulation cause bronchi spasm
B. Secretomotor to the mucous glands of the bronchial tree
C. Sensory fibres are responsible for the stretch reflex of the lungs and for the cough reflex
-Sympathetic nerves derived from second to fifth sympathetic ganglia. These are the inhibitory
to the smooth muscles and glands of the bronchial tree. 7

10
Fig.7.Nerve Innervation of lungs

11
Fig.8.Nerve supply of lungs

12
 PHYSIOLOGY
The lungs are the foundational organs of the respiratory system, whose most basic function is
to facilitate gas exchange from the environment into the bloodstream. Oxygen gets transported
through the alveoli into the capillary network, where it can enter the arterial system, ultimately
to perfuse tissue. The respiratory system is composed primarily of the nose, oropharynx, larynx,
trachea, bronchi, bronchioles and lungs. The lungs further divide into individual lobes, which
ultimately subdivide into over 300 million alveoli. The alveoli are the primary location for gas
exchange. The diaphragm is the primary respiratory muscle and receives innervation by the
nerve roots of C3, C4, and C5 via the phrenic nerve. The external intercostals are inspiratory
muscles used primarily during exercise and respiratory distress. The significant lung
volumes/capacities and their definitions are listed below.
Inspiratory reserve volume (IRV): Volume that can be breathed after a normal inspiration
Tidal volume (TV): Volume inspired and expired with each breath
Expiratory reserve volume (ERV): Volume that can be expired after a normal breath
Residual volume (RV): Volume remaining in lung after maximal expiration (cannot be
measured by spirometry)
Inspiratory capacity (IC): Volume that can be breathed after normal exhalation
Functional residual capacity (FRC): Volume remaining in the lungs after normal expiration
Vital capacity (VC): Maximum volume able to be expired after maximal inspiration
Total lung capacity (TLC): Volume of air in the lungs after maximal inspiration
Forced expiratory volume (FEV1): Volume that can be expired in 1 second of maximum forced
expiration.
Successful exchange of gases in the lungs is predicated on efficient ventilation of the lungs.
Tidal respiration is based on cyclical expansion and contraction of the lungs. During inhalation,
active contraction of the diaphragm and external intercostal muscles of the ribcage draw air
into the lungs. Each of these muscle groups acts together to expand the lungs. During
inhalation, intrathoracic pressures decrease and intraabdominal pressures increase.
Oxygen transport is the primary means by which the circulatory system perfuses tissue. Oxygen
gets carried in the body in two major forms: bound to haemoglobin and dissolved.
Haemoglobin is the major oxygen carrier in the body. The formula for the oxygen content of
blood is as follows:

CaO2 = 1.34 x [Hgb] x (SaO2 / 100) + 0.003 x PaO2

CaO2 = oxygen content in blood
[Hb] = hemoglobin concentration
SaO2 = percentage of heme groups that are bound to oxygen
PaO2 = Partial pressure of oxygen

13
Four subunits comprise hemoglobin, each containing a heme-moiety that binds to iron. One
molecule of O2 can bind to each iron atom of the heme group; therefore, each hemoglobin
group can bind to four molecules of O2.

Development
Lung development in-utero occurs in five main stages. The first stage begins with the
development of the lung bud from the respiratory diverticulum during week 4 of
embryogenesis. The stages are as follows

Embryonic: begins from weeks 4 to 7; this is when the formation of the major airways and
pleura occur.
Psuedoglandular: occurs during weeks 5 to 17; this is when the bronchial tree and respiratory
parenchyma form.
Canalicular: occurs during weeks 16 to 26; the distal airway, blood-air barrier, surfactant, and
acini form.
Saccular: occurs during weeks 24 to 38 weeks; the airspaces (alveoli) continue to expand.
Alveolar: occurs from week 36 of gestation and throughout childhood. The alveoli become
septated and more mature - improving airspace and capillary networks 8.

FUNCTION
The function of the pulmonary system is to extract oxygen from the environment and provide
it for aerobic respiration at the cellular level. Oxygen is ultimately used to produce ATP, and
carbon dioxide is breathed out with other metabolic by products.
Respiratory tract organs facilitate the process of gas exchange, including the nose, oral cavity,
throat, trachea, bronchi, and lungs. The lungs divide into five major lobes: three lobes on the
right and two lobes on the left. Each lobe is made up of many small alveoli, which are the
primary site of gas exchange. At the alveoli, diffusion of gases into the arterioles occurs.
RESPIRATORY SYSTEM FUNCTIONS
Supplies the body with oxygen and disposes of carbon Dioxide
-Filters inspired air
-Produces sound
-Clears the body from excess water and heat
- Control blood pH

14
RESPIRATORY EVENTS
Pulmonary ventilation=exchange of gases between lungs and atmosphere
External respiration= exchange of gases between alveoli and pulmonary capillaries
Internal respiration= exchange of gases between systemic capillaries and tissue cells.

PHASES OF PULMONARY VENTILATION

Inspiration, or inhalation- A very active process that requires input of energy. The diaphragm
contracts , moving downward and flattening
Expiration or exhalation-A passive process that takes advantage of the recoil properties of
elastic fiber. The diaphragm relaxes. The elasticity of the lungs and the thoracic cage allows
them to return to their normal size and shape
This two processes are happens when phrenic nerves stimulates.

INHALATION
Breathing in is called inhalation (inspiration).
Each inhalation, the air pressure inside the lungs is equal to air pressure of the atmosphere,
which is about 760mmHg.
Air to flow into the lungs, the pressure inside the alveoli must become lower than the
atmospheric pressure.
This condition is achieved by increasing the size of lungs.

EXHALATION
It is the diffusion of Oxygen from air in the alveoli of the lungs to blood in pulmonary
capillaries and the diffusion of carbondioxide in the opposite direction.
External respiration in the lungs converts deoxygenated blood coming from the right side of
the heart into oxygenated blood that returns to the left side of the heart.
As blood flows through the pulmonary capillaries, it picks up oxygen from alveolar air and
unloads carbondioxide into alveolar air this process is called an exchange of gases, this process
is carried by diffusion

15
OXYGEN TRANSPORT
Oxygen does not dissolve easily in water, so only about 1.5% of inhaled oxygen is dissolved
in blood plasma, which is mostly water. About 98.5% of blood oxygen is bound to haemoglobin
in red blood cells. Each 100mL of oxygenated blood contains the equivalent of 20mL of
gaseous oxygen.
The heme portion of haemoglobin contains four atoms of iron, each capable of binding to a
molecule of oxygen. There 98.5% of the oxygen is bound to haemoglobin. Oxygen and
haemoglobin bind in an easily reversible reaction to form oxyhemoglobin.
As blood flows through tissue capillaries, the iron-oxygen reaction reverses. Haemoglobin
releases oxygen, which diffuses first into interstitial fluid and then into cells.

Fig.9.Oxygen transport

16
REGULATION OF RESPIRATION
Regulation of respiration control is the rate and depth of respiration as per the physiologic
demand. Control of respiration primarily involves neurons in the reticular formation of medulla
and pons. Because the medulla sets the respiratory rhythm. The purpose of regulation of
respiration are
.To maintain a constant oxygen and carbondioxide level in blood, it adjust the oxygen supply
as per metabolic demand of the body, and it helps to regulate acid base balance or pH.
The size of the thorax is altered by the action of the respiratory muscles which contract as a
result of nerve impulses transmitted to them from centers in the brain and relaxes in the absence
of nerve impulses. This impulses travels along the phrenic nerve and intercostal nerves to excite
the diaphragm and external intercostal muscles.
These nerve impulses are sent from clusters of neurons located bilaterally in the medulla
oblongata and pons of the brain stem.
This widely dispersed group of neurons, collectively called the respiratory center 9.
MECHANISM OF REGULATION OF RESPIRATION
There are two major mechanisms
-Nervous regulation of respiration
-Chemical regulation of respiration

NERVOUS REGULATION OF RESPIRATION

Respiratory center
The respiratory centers are divided into four major groups, two groups in the medulla and two
groups in pons.
The two groups in the medulla are
1 The dorsal respiratory group
2. The ventral respiratory group
The two groups in the pons are the pneumotaxic centre and apneustic center also known the
pontine respiratory group.
Respiratory center can be divided into three areas on the basis of their functions
1. The medullary rhythmically area in the medulla oblongata
2. The pneumotaxic area in the pons
3. The apneustic area also in the pons

17
APNEUSTIC AREA
This area sends stimulatory impulses to the inspiratory area that activate it and prolong
inhalation. The result is a long, deep inhalation.

CHEMICAL REGULATION OF RESPIRATION

There are three important chemical factors controlling respiration
-Concentration of carbondioxide in blood
-Concentration of H+ ions or pH
-Concemtration of oxygen in blood 10

Fig. 10.Chemical regulation of respiration

18
Fig.11.Pathway of air through respiratory system

19
 PATHOPHYSIOLOGY
The intrinsic pulmonary restriction is most commonly characterized by inflammation of the
pulmonary parenchyma with subsequent deposition of collagen in the interstitium, which
ultimately leads to pulmonary fibrosis. Over time, this progressive fibrosis thickens the alveolar
septae, imposing a physical barrier to gas exchange. This is reflected in pulmonary function
testing (PFT), where decreased diffusing capacity for carbon monoxide (DLCO) can be
observed. Besides decreasing DLCO, lung tissue fibrosis decreases the compliance of the lung
tissue, reducing the inspiratory capacity.
The pathophysiology of extrapulmonary forms provides an altered mechanical function very
often induced by deformations of the rib cage, but also linked to neurological or muscular
pathologies, or from the occupation of space, as in the case of pleural effusions 11
The Compliance
The distensibility of the respiratory system is termed as compliance (C). It a key concept in
respiratory physiology and represents the volume change produced by a change in the
distending pressure. It is determined by elastic forces formula:
C=ΔV/ΔP
Of note, lung compliance is independent of the thoracic cage, which is a semirigid casing, but
lung and thorax are systems arranged in series. Thus, the compliance of an intact respiratory
system is the sum of the compliances of both of these structures. Therefore, it is affected by
any disease of the lungs, pleura, or chest wall. Lung compliance decreases if the lung is over
rigid (restrictive pathologies) and increases in less stiff conditions such as emphysema.
Thoracic compliance decreases in conditions of reduced distensibility of the rib cage (obesity,
kyphoscoliosis). Combined compliance of thorax and lungs is 110 ml/cmH2O, whereas the
compliance of lung alone is approximately 200 ml/cmH2O. Decreased compliance, either from
intrapulmonary or extra pulmonary etiology, results in greater pressure generation and energy
requirements from the respiratory muscles for any given tidal volume. These changes in
respiratory physiology eventually lead to tissue hypoxia and dyspnea.
Hypoxemia:
Hypoxemia (PaO2<60 mmHg), whether or not in association with hypercapnia (PaCO2>45
mmHg), can be a consequence of lung damage, and an expression of respiratory failure. In
restrictive syndromes, it occurs with different mechanisms depending on the type of the
disease. In the intrinsic conditions, it is produced by alterations in the gas diffusion with a
mismatch in the ventilation-perfusion ratio. This process leads, in turn, to an increased
intrapulmonary shunt that is the portion of the pulmonary blood flow that bypasses the alveoli
or perfuses unventilated alveoli and does not participate in the gas exchanges. On the other
hand, hypoventilation and pump failure are prominent in the extrinsic conditions 12.
Functional Characteristics:
Functional characteristics of a restrictive pattern in pulmonary function tests (PFTs) include
decreased TLC and forced vital capacity (FVC). This latter parameter is the maximum amount
of air exhaled after a maximal inhalation and depends on the elasticity of the lung tissue, the
anatomy of the thoracic cage, and the function of the respiratory muscles. According to the

20
American Thoracic Society (ATS), the TLC predicted (adjusted for gender, age, height) value
can be used for assessing the severity of the restrictive condition:
TLC < 80% = Restrictive disease
70-80 % predicted: mild restrictive disease
60-70% predicted: moderate restrictive disease
50-60% predicted: moderately severe restrictive disease
< 50% predicted: severe restrictive disease
In restrictive diseases, the forced expiratory volume over 1 second (FEV1) usually slightly
decreases or stays normal, and the ratio of FEV1 to FV
C is generally preserved or increased. These alterations in pulmonary dynamics can be
compensated by an increased respiratory rate, with hypercapnia developing only at later stages
of the disease.
Restrictive Patterns:
-Decreased FVC and TLC, FEV1 is usually slightly decreased or stays normal, the ratio of
FEV1 to FVC is usually preserved or increased, Reduced diffusing capacity for carbon
monoxide (DLCO).
Diffuse parenchymal disorders cause reduction in all lung volumes. This is produced by
excessive elastic recoil of the lungs.
Expiratory flows are reduced in proportion to lung volumes. Arterial hypoxemia is caused by
ventilation/perfusion mismatch.
Impaired diffusion of oxygen will cause exercise induced desaturation. Hyperventilation at rest
secondary to reflex stimulation.
As some diseases of the lung parenchyma progress, the normal lung tissue can be gradually
replaced with scar tissue that is interspersed with pockets of air. This can lead to parts of the
lung having a honeycomb-like appearance. The extrinsic causes result in lung restriction,
impaired ventilatory function, and even respiratory failure due to the diseases that effect the
lungs ability to create a change in lung volumes during respiration due to the diseases of the
systems stated above 13.

21
 CLASSIFICATION
It can be mainly classified into two types depending on whether the cause is intrinsic or
extrinsic.
Pulmonary parenchyma ,.Extra pulmonary
PULMONARY PARENCHYMA (INTRINSIC)
Intrinsic restrictive lung diseases affect the functional tissue of the lung, including the alveoli,
capillaries, and the interstitium. The alveoli are small branches of air tubes in the lungs.
Capillaries are blood vessels, and the interstitium refers to a group of tissues within the lung
that provide support.
Intrinsic restrictive lung diseases usually result from inflammation and scarring of lung tissue.
The cause may be interstitial lung disease. This is an umbrella term for a large group of diseases
that cause scarring of the lungs. The scarring leads to stiffness that makes it difficult to breathe
and get oxygen to the bloodstream. Damage from interstitial lung disease is often irreversible
and gets worse over time.
One example of intrinsic restrictive lung disease is idiopathic pulmonary fibrosis. Some other
health issues that may be involved in intrinsic restrictive lung disease include:
-sarcoidosis:
Sarcoidosis is a disease characterized by the growth of tiny collections of inflammatory cells
(granulomas) in any part of your body — most commonly the lungs and lymph nodes. But it
can also affect the eyes, skin, heart and other organs.
-Connective tissue diseases, such as scleroderma:
Scleroderma (sklair-oh-DUR-muh), also known as systemic sclerosis, is a group of rare
diseases that involve the hardening and tightening of the skin. It may also cause problems in
the blood vessels, internal organs and digestive tract.
-Drug-induced lung disease:
Due to this, it can be extremely difficult to pinpoint the offending agent on imaging appearances
alone and correlation with the medical history is mandatory.
-Exposure to toxins:
Given the continuous exposure of the upper and lower airways and lung parenchyma to the
environment, the possibility that we may inhale any of a broad array of toxic substances is not
surprising. Several factors are important in determining the location and magnitude of toxic
lung injury that arises from inhalation of smoke, aerosols, vapors, or fumes, including the size
and water solubility of the inhaled material 14

22
EXTRAPULMONARY (EXTRINSIC)
Extrinsic restrictive lung diseases involve the chest wall, pleura, and respiratory muscles. The
pleura is a membrane that covers the inside surface of the rib cage and spreads over the lungs.
Diseases of these structures result in lung restriction, impaired function, and respiratory failure.
Neuromuscular disorders can be extrinsic restrictive lung diseases. Some examples include
multiple sclerosis, muscular dystrophy, and amyotrophic lateral sclerosis, better known as
ALS.
Some other health issues that can be involved in extrinsic restrictive lung disease include:
-Pleural effusion:
Pleural effusion is the accumulation of fluid in between the parietal and visceral pleura, called
the pleural cavity. It can occur by itself or can be the result of surrounding parenchymal disease
like infection, malignancy or inflammatory conditions. Pleural effusion is one of the major
causes of pulmonary mortality and morbidity.
-Scoliosis, or twisting of the spine:
Severe scoliosis may have a significant effect on respiratory function. The effect is most often
restrictive due to severe anatomical distortion of the chest, leading to reduced lung volumes,
limited diaphragmatic excursion and chest wall muscle inefficiency.
-Obesity:
Obesity causes mechanical compression of the diaphragm, lungs, and chest cavity, which can
lead to restrictive pulmonary damage. Furthermore, excess fat decreases total respiratory
system compliance, increases pulmonary resistance, and reduces respiratory muscle strength.
-Myasthenia gravis, or intermittent muscle weakness:
Myasthenia gravis can affect the respiratory system, causing respiratory muscle weakness, an
abnormal breathing pattern, and blunted ventilatory responses.
-Rib damage, especially fractures:
A serious rib fracture can damage the nearby internal organs, nerves, or blood vessels. The
sharp end of a displaced broken rib may puncture the lung.
-Diaphragm paralysis:
The diaphragm contracts downward to cause a negative intrathoracic pressure to allow lung
expansion and passive air flow into the lungs for gas exchange. Unilateral diaphragmatic
paralysis is a condition in which either the right or left side of the diaphragm loses the ability
to contract to allow proper inspiration. This can result from muscular issues in the diaphragm
or loss of innervation from the phrenic nerve to the hemidiaphragm. This can impact daily
living by causing dyspnea at rest or during exercise, sleep disturbances, or in severe cases,
decrease life expectancy.

23
-Kyphosis, or hunching of the upper back:
Hyperkyphosis reduces the amount of space in the chest, mobility of the rib cage, and
expansion of the lungs. Decline in pulmonary function may be greater in persons with more
severe kyphosis 15

Fig.12.Representation of restrictive lung

24
 ETIOLOGY
The acronym "PAINT" can be used to memorize the causes of restrictive lung disease into
pleural, alveolar, interstitial, neuromuscular, and thoracic cage abnormalities. Nevertheless, a
more accurate division takes into account the pathogenetic mechanism 16.
Restrictive lung syndromes can be caused by:
 Pulmonary parenchyma diseases (intrinsic causes)
 Extrapulmonary diseases (extrinsic causes)
Intrinsic, or pulmonary causes, involve the lung parenchyma itself, while the extrinsic
restrictive lung diseases originate from neuromuscular disorders, obesity, and other extra-
parenchymal disorders. In both intrinsic and extrinsic pulmonary conditions, lung volumes
become reduced due to restrictions in pulmonary mechanics.
Pulmonary Parenchyma Diseases (Intrinsic Causes)
Intrinsic restriction can be caused by intrapulmonary restriction due to inflammatory processes
within the lung tissue by diseases categorized under interstitial lung diseases.
 Idiopathic pulmonary fibrosis (IPF)
 Non-specific interstitial pneumonia (NSIP)
 Cryptogenic organizing pneumonia (COP)
 Sarcoidosis
 Acute interstitial pneumonia (AIP) 17
Inorganic dust exposure such as silicosis, asbestosis, talc, pneumoconiosis, berylliosis, hard
metal fibrosis, coal worker's pneumoconiosis, chemical worker's lung
Organic dust exposure such as farmer's lung, bird fancier's lung, bagassosis, and mushroom
worker's lung, humidifier lung, hot tub pneumonitis
 Hypersensitivity pneumonitis
 Systemic sclerosis
 Pulmonary vasculitis
 Pulmonary Langerhans cell histiocytosis (formerly referred to as histiocytosis X)
 Medications such as nitrofurantoin, amiodarone, gold, phenytoin, thiazides,
hydralazine, bleomycin, carmustine, cyclophosphamide, methotrexate
 Radiation therapy
These conditions can be grouped into diseases that lead to an increased elastic return (e.g.,
diffuse infiltrative pneumopathies, and interstitial diseases), and diseases
Provoked by the occupation of alveolar spaces (e.g., pneumonia) 18.
Extrinsic or Extrapulmonary Diseases
They can be a result of diseases of the chest wall, such as:
 Kyphoscoliosis

25
  Pleural conditions such as effusions, trapped lung, pleural scarring, chronic empyema,
asbestosis
 Obesity
 Neuromuscular disorders like muscular dystrophy, amyotrophic lateral sclerosis, polio,
and phrenic neuropathies.
 Ascites
Extrapulmonary diseases can also be schematically grouped into pathologies that produce a
decreased muscle tone of the respiratory pump (e.g., myopathies, and neurological deficits),
deformations of the rib cage (e.g., kyphoscoliosis), and space occupation (e.g., pleural
effusions, pneumothorax) 19.
In brief, the causes of restrictive diseases can, therefore, be manifold. In some conditions, such
as IPF, the causes are unknown. In this context, a challenge concerns the genetic factors that
contribute to disease risk. For instance, illustrated the genetics in IPF and correlations with
prognosis and treatment. More recently, the role of rare variation in FAM13A, TERT, and
RTEL1 gene regions involved in the risk of IPF was demonstrated 20.

26
 CLINICAL FEATURES
Each condition has its own set of signs and symptoms which are unique to the particular disease
and its pathophysiology. However, most are characterized by an insidious progression of
dyspnea. Moreover, ILDs classically produce the "3Cs": cough, clubbing of the nails, and
coarse crackles on auscultation 21.
Common additional characteristics in patients with extrinsic restriction will include an
increased BMI, spine deviation, or have a history of neuromuscular disease.
Clinical features and prognosis vary among the diseases. For instance, IPF involves a
progressive decline in lung function that leads to eventual respiratory failure. As a
consequence, the condition has a poor prognosis with a median survival of 3 to 5 years after
diagnosis.

The symptoms of restrictive lung disease include:

 shortness of breath
 wheezing
 coughing
 chest pain
 Fatigue and weakness
 Loss of appetite
 Loss of weight
 Dry cough that does not produce phlegm
 Discomfort in the chest
 Labored breathing 22

27
 RISK FACTORS

There are factors that can increase risk of developing restrictive lung diseases:
Smoking:
A history of smoking cigarettes or other tobacco products increases the risk of developing
restrictive lung diseases
Workplace Exposure:
People who have worked in certain industries, such as in construction, factory work, or
farming, may have had exposure to certain chemicals or other compounds that increase the
risk. These chemicals and compounds can include:
-Asbestos
-Mold
-Heavy amounts of dust
-Wood dust
-Silica
-Animal feed
-Bird droppings or livestock waste
-Coal
*Working in well-ventilated areas or wearing properly fitting respiratory masks can help
decrease the number of particles inhaled.
Medical Risk Factors:
In addition to the workplace exposures outlined above, there are other risk factors associated
with the development of restrictive lung diseases. They are:
Medications: Certain medications can increase risk, including amiodarone, some
chemotherapy medications, and methotrexate.
Radiation: Previous exposure to radiation can increase the risk 23

28
 INVESTIGATIONS
Diagnostic testing for lung disease may include any of the following:

Fig. 13. (Image: Spirometry is one of the pulmonary function tests)

-Chest X-Ray
The most common radiographic abnormality is a reticular pattern. Nodular, reticulonodular,
or mixed patterns, such as alveolar filling (i.e., ground-glass appearance).
-CT scans
High-resolution CT scanning of the chest can be helpful, but the expense and high dose of
radiation makes it inappropriate for every patient. IPF can be diagnosed clinically based on
the typical clinical features and CT scan findings without the need for lung biopsy.
-Bronchoscopy
A bronchoscope is a device used to see the inside of the airways and lungs. The scope can be
flexible or rigid. A flexible scope is almost always used. It is a tube less than one half inch (1
centimetre) wide and about 2 feet (60 centimetres) long. In rare cases, a rigid bronchoscope is
used.
-Pulse oximeter
Pulse oximetry is a non-invasive test that measures the oxygen saturation level of your
blood. It can rapidly detect even small changes in oxygen levels. These levels show how
efficiently blood is carrying oxygen to the extremities furthest from your heart, including your
arms and legs.
-Pulmonary function test:
The mainstay for evaluation for pulmonary restriction is based on pulmonary function tests
(PFTs). Initial results indicative of pulmonary restriction will be a decreased TLC with a
preserved FEV1/FVC ratio (greater than 70%). Once restrictive, a restrictive pattern is
suggested on spirometry; patients should be referred for full PFTs with DLCO measurement,
which will be decreased on patients with intrinsic pulmonary restriction. Patients with extrinsic
pulmonary restriction will also present with a restrictive pattern on PFTs; however, DLCO

29
remains normal. Additional testing with high resolution computed tomography (HRCT),
inflammatory markers, and specific autoantibodies should be done on an individual basis
according to the suspected etiology of the pulmonary restriction.
In the IPF, the high-resolution computerized tomography (HRTC) demonstrates the typical
appearance with coarse crosslinking in the basal regions, posterior in the early stages,
peripheral/sub-pleural. "Honeycomb" cystic alterations and bronchiectasis that become evident
for traction fibrotic 24.

30
 MANAGEMENT
Treatment of lung disease depends on many factors, such as the type and stage of disease,
family history, patient’s medical history and the health and age of the patient. Any of the
following may be used for treating lung disease
MEDICAL MANAGEMENT
-Inhalers, Expectorants, Antibiotics, Corticosteroids.
Oxygen therapy: The main treatment for restrictive lung disease is supportive oxygen therapy.
Oxygen therapy helps people with lung diseases get enough oxygen, even when their lungs
cannot fully expand. Some people may need oxygen only at night or after exerting themselves.
Others need oxygen all or most of the time.
Chemotherapy: may help reduce inflammation caused by a restrictive lung disease. However,
chemotherapy also kills healthy cells and can prompt a range of side effects, such as nausea,
hair loss, and a weakened immune system. Some studies Trusted Source have also found a link
between chemotherapy and lung damage. As such, it is important to weigh the potential
benefits of chemotherapy against its risks 25.
SURGICAL MANAGEMENT
Lung transplantation: Lung transplants offer a chance of a healthier, longer life, but they are
also highly risky. After a lung transplant, a person can develop life-threatening complications,
such as organ rejection. After a lung transplant, a recipient must take drugs that suppress the
immune system. However, these drugs make transplant recipients more vulnerable to many
infections. As a result, most transplant recipients must be closely monitored in hospital for
several weeks.
PHYSIOTHERAPY MANAGEMENT
Pulmonary Rehabilitation program will cover the following core educational topics: managing
breathlessness and cough, overcoming fatigue, managing anxiety, depression and panic, basics
of oxygen therapy, and maintenance of physical activity after pulmonary rehabilitation.
Physiotherapists play an important role in prescribing, supervising, in exercise. There are
various physiotherapy treatments incorporated within chest physiotherapy, breath conditioning
techniques, such as pursed-lip breathing, slow, deep breathing, or diaphragmatic breathing
upper and lower limb strengthening and conditioning exercises respiratory muscle
strengthening exercises ,level walking.

31
RECENT ADVANCES
Stem cell therapy is still an experimental treatment, but for some people with restrictive lung
disease, it may offer the chance of a longer life.
Clinical trials: Treatment for restrictive lung disease is continually evolving. Some people with
symptoms of the disease may be eligible for a clinical trial. There is no guarantee that new
drugs will work, but many clinical trials have helped people get better.
Impairment in pulmonary function in patients with severe scoliosis may be controlled with
surgical correction.
For obese patients, management involves losing weight by a combination of diet and physical
exercise. Morbidly obese patients who fail to lose weight by traditional methods should be
referred for gastric bypass surgery evaluation.

Ventilator therapy: A ventilator is a device that helps the lungs take in oxygen. Unlike oxygen
therapy that is given through the nose, a ventilator uses a tube in the throat or a high-pressure
mask to support breathing. People who have an advanced restrictive lung disease may need a
ventilator to be able to breathe.
For some people, ventilator therapy is unsafe or cannot be used. Hence, in extreme cases, an
alternative called extracorporeal membrane oxygenation (ECMO) delivers oxygen directly to
the blood. For ECMO, a doctor removes blood through a large vein. The blood is pumped
through a membrane that provides it with oxygen, and it is then put back in the body 26.

32
 SARCOIDOSIS
Sarcoidosis (also known as Besnier-Boeck-Schaumann disease) is a disease involving
abnormal collections of inflammatory cells that form lumps known as granulomata. The disease
usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver,
heart, and brain. Any organ, however, can be affected. The signs and symptoms depend on the
organ involved. Often, no, or only mild, symptoms are seen. When it affects the lungs,
wheezing, coughing, shortness of breath, or chest pain may occur. Some may have Löfgren
syndrome with fever, large lymph nodes, arthritis, and a rash known as erythema nodosum.
Sarcoidosis is a potentially life-threatening disease with no known cause or cure. The duration
of this disease varies and can last from only a few short months to several years. Often times,
sarcoidosis is self-limiting needing no medicinal treatment. The primary manifestation of
sarcoidosis includes inflammation of the lungs as well as many other bodily organs. About
90% of diagnosed cases are deemed to be pulmonary sarcoidosis. Results include stiffness of
the lungs due to scar tissue formation causing decreased oxygen uptake and gas exchange in
the bloodstream. Other involvement of sarcoidosis can manifest itself as inflammation of the
epithelial tissue, eyes, liver, spleen, heart, kidneys, central nervous system (CNS) and bones of
the hands or feet. Although this disease has not always been well known, cases have been
recorded worldwide and are becoming increasingly more common.

EPIDEMIOLOGY
Sarcoidosis occurs throughout the world in all races with an average incidence of 16.5 per
100,000 in men and 19 per 100,000 in women. The disease is most common in Northern
European countries and the highest annual incidence of 60 per 100,000 is found in Sweden and
Iceland. In the United Kingdom the prevalence is 16 in 100,000.In the United States,
sarcoidosis is more common in people of African descent than Caucasians, with annual
incidence reported as 35.5 and 10.9 per 100,000, respectively. Sarcoidosis is less commonly
reported in South America, Spain, India, Canada, and the Philippines. The incidence across the
world may be at least partially attributable to the lack of screening programs in certain regions
of the world, and the overshadowing presence of other granulomatous diseases, such as
tuberculosis, that may interfere with the diagnosis of sarcoidosis where they are prevalent.
There may also be differences in the severity of the disease between people of different
ethnicities. Several studies suggest the presentation in people of African origin may be more
severe and disseminated than for Caucasians, who are more likely to have asymptomatic
disease. Manifestation appears to be slightly different according to race and sex. Erythema
nodosum is far more common in men than in women and in Caucasians than in other races. In
Japanese people, ophthalmologic and cardiac involvement are more common than in other
races. 27

PATHOPHYSIOLOGY
Granulomatous inflammation is characterized primarily by the accumulation of macrophages
and activated T-lymphocytes, with increased production of key inflammatory mediators,
tumour necrosis factor alpha (TNF), interferon gamma, interleukin 2 (IL-2), IL-8, IL-10, IL-

33
12, IL-18, IL-23 and transforming growth factor beta (TGF-β), indicative of a T helper cell-
mediated immune response. Sarcoidosis has paradoxical effects on inflammatory processes; it
is characterized by increased macrophage and CD4 helper T-cell activation, resulting in
accelerated inflammation, but immune response to antigen challenges such as tuberculin is
suppressed. This paradoxic state of simultaneous hyper- and hypo activity is suggestive of a
state of energy. The energy may also be responsible for the increased risk of infections and
cancer. [Citation needed] The regulatory T-lymphocytes in the periphery of sarcoid granulomas
appear to suppress IL-2 secretion, which is hypothesized to cause the state of anergy by
preventing antigen-specific memory responses.
While TNF is widely believed to play an important role in the formation of granulomas (this is
further supported by the finding that in animal models of mycobacterial granuloma formation
inhibition of either TNF or IFN-γ production inhibits granuloma formation), sarcoidosis can
and does still develop in those being treated with TNF antagonists like etanercept. [95] B cells
also likely play a role in the pathophysiology of sarcoidosis. Serum levels of soluble human
leukocyte antigen (HLA) class I antigens and angiotensin converting enzyme (ACE) are higher
in people with sarcoidosis. Likewise the ratio of CD4/CD8 T cells in bronchoalveolar lavage
is usually higher in people with pulmonary sarcoidosis (usually >3.5), although it can be normal
or even abnormally low in some cases. Serum ACE levels have been found to usually correlate
with total granuloma load.
CLASSIFICATION:
Sarcoidosis may be divided into the following types:
-Annular sarcoidosis
Annular sarcoidosis is a cutaneous condition characterized by papular skin lesions arranged in
annular patterns, usually with a red-brown hue.
-Erythrodermic sarcoidosis
Erythrodermic sarcoidosis is a cutaneous condition and very rare form of sarcoidosis.
-Ichthyosiform sarcoidosis
Ichthyosiform sarcoidosis is a cutaneous condition resembling ichthyosis vulgaris or acquired
ichthyosis, with fine scaling usually on the distal extremities, by caused by sarcoidosis.
-Hypo pigmented sarcoidosis
Hypopigmented sarcoidosis is a cutaneous condition characterized by areas of hypopigmented
skin. It is usually diagnosed in darkly pigmented races and may be the earliest sign of
sarcoidosis.
-Löfgren syndrome
Löfgren syndrome is a type of acute sarcoidosis, an inflammatory disorder characterized by
swollen lymph nodes in the chest, tender red nodules on the shins, fever and arthritis.
-Lupus pernio

34
Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color.
It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.
-Morpheaform sarcoidosis
Morpheaform sarcoidosis is a very rare cutaneous condition characterized by specific
cutaneous skin lesions of sarcoidosis accompanied by substantial fibrosis, simulating morphea.
-Mucosal sarcoidosis
Mucosal sarcoidosis is a cutaneous condition characterized by pinhead-sized papules that may
be grouped and fused together to form a flat plaque.
-Neurosarcoidosis
In this type involving the central nervous system (brain and spinal cord). Neurosarcoidosis can
have many manifestations, but abnormalities of the cranial nerves (a group of twelve nerves
supplying the head and neck area) are the most common.
-Papular sarcoid
Papular sarcoid is a cutaneous condition characterized by papules, which are the most common
morphology of cutaneous sarcoidosis.
-Scar sarcoid
Scar sarcoid (also known as "Sarcoidosis in scars") is a cutaneous condition characterized by
infiltration and elevation of tattoos and old flat scars due to sarcoidosis.
-Subcutaneous sarcoidosis
Subcutaneous sarcoidosis (also known as "Darier–Roussy disease" and "Darier-Roussy
sarcoid") is a cutaneous condition characterized by numerous 0.5- to 0.3-cm deep-seated
nodules on the trunk and extremities.
-Systemic sarcoidosis
The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the
eyes, liver, heart, and brain. Any organ, however, can be affected
-Ulcerative sarcoidosis
Ulcerative sarcoidosis is a cutaneous condition affecting roughly 5% of people with
sarcoidosis.
ETIOLOGY
Sarcoidosis has no known cause. However, some researchers believe the course of the disease
may have genetic connections or result from an immune response. Others possible causes
include viruses, bacteria’s, dust particles, or chemical exposure. Due to the vast nature of the
manifestation, other researchers have led one to believe that there may be several causes.
Findings that make it likely include large lymph nodes at the root of the lung on both sides,
high blood calcium with a normal parathyroid hormone level, or elevated levels of angiotensin-

35
converting enzyme in the blood. The diagnosis should only be made after excluding other
possible causes of similar symptoms such as tuberculosis.
Genetics:
The heritability of sarcoidosis varies according to ethnicity. About 20% of African Americans
with sarcoidosis have a family member with the condition, whereas the same figure for
European Americans is about 5%. Additionally, in African Americans, who seem to experience
more severe and chronic disease, siblings and parents of sarcoidosis cases have about a 2.5-
fold increased risk for developing the disease. In Swedish individuals heritability was found to
be 39%. In this group, if a first-degree family member was affected, a person has a four-fold
greater risk of being affected.
Investigations of genetic susceptibility yielded many candidate genes, but only few were
confirmed by further investigations and no reliable genetic markers are known. Currently, the
most interesting candidate gene is BTNL2; several HLA-DR risk alleles are also being
investigated. In persistent sarcoidosis, the HLA haplotype HLA-B7-DR15 is either cooperating
in disease or another gene between these two loci is associated. In nonpersistent disease, a
strong genetic association exists with HLA DR3-DQ2. Cardiac sarcoid has been connected to
tumour necrosis factor alpha (TNFA) variants.
Infectious agents:
Several infectious agents appear to be significantly associated with sarcoidosis, but none of the
known associations is specific enough to suggest a direct causative role. The major implicated
infectious agents include: mycobacteria, fungi, borrelia, and rickettsia. A meta-analysis
investigating the role of mycobacteria in sarcoidosis found it was present in 26.4% of cases,
but they also detected a possible publication bias, so the results need further confirmation.
Mycobacterium tuberculosis catalase-peroxidase has been identified as a possible antigen
catalyst of sarcoidosis. The disease has also been reported by transmission via organ
transplants. A large epidemiological study found little evidence that infectious diseases
spanning years before sarcoidosis diagnosis could confer measurable risks for sarcoidosis
diagnosis in the future.
Autoimmune:
Association of autoimmune disorders has been frequently observed. The exact mechanism of
this relation is not known, but some evidence supports the hypothesis that this is a consequence
of Th1 lymphocyte prevalence. Tests of delayed cutaneous hypersensitivity have been used to
measure progression .

36
SYMPTOMS
Sarcoidosis is a systemic inflammatory disease that can affect any organ, although it can be
asymptomatic and is discovered by accident in about 5% of cases. Common symptoms, which
tend to be vague, include fatigue (unrelieved by sleep; occurs in up to 85% of cases , lack of
energy, weight loss, joint aches and pains (which occur in about 70% of cases), arthritis (14–
38% of cases), dry eyes, swelling of the knees, blurry vision, shortness of breath, a dry, hacking
cough, or skin lesions. Less commonly, people may cough up blood. Sarcoidosis is also
accompanied by psychological distress and symptoms of anxiety and depression, which are
also associated with fatigue. The cutaneous symptoms vary, and range from rashes and nodule
(small bumps) to erythema nodosum, granuloma annulare, or lupus pernio. Sarcoidosis and
cancer may mimic one another, making the distinction difficult.
The combination of erythema nodosum, bilateral hilar lymphadenopathy, and joint pain is
called Löfgren syndrome, which has a relatively good prognosis. This form of the disease
occurs significantly more often in Scandinavian patients than in those of non-Scandinavian
origin.
Respiratory tract:
Localization to the lungs is by far the most common manifestation of sarcoidosis. At least 90%
of those affected experience lung involvement. Overall, about 50% develop permanent
pulmonary abnormalities, and 5 to 15% have progressive fibrosis of the lung parenchyma.
Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory
process involves the alveoli, small bronchi, and small blood vessels. In acute and subacute
cases, physical examination usually reveals dry crackles. At least 5% of cases include
pulmonary arterial hypertension. The upper respiratory tract (including the larynx, pharynx,
and sinuses) may be affected, which occurs in between 5 and 10% of cases.
The four stages of pulmonary involvement are based on radiological stage of the disease, which
is helpful in prognosis:
Stage I: Bilateral hilar lymphadenopathy (BHL) alone
Stage II: BHL with pulmonary infiltrates
Stage III: pulmonary infiltrates without BHL
Stage IV: fibrosis
Use of the Scadding scale only provides general information regarding the prognosis of the
pulmonary disease over time. Caution is recommended, as it only shows a general relation with
physiological markers of the disease and the variation is such that it has limited applicability
in individual assessments, including treatment decisions. Use of the Scadding scale only
provides general information regarding the prognosis of the pulmonary disease over time.
Caution is recommended, as it only shows a general relation with physiological markers of the
disease and the variation is such that it has limited applicability in individual assessments,
including treatment decisions.

37
Skin:
Sarcoidosis involves the skin in between 9 and 37% of cases and is more common in African
Americans than in European Americans. The skin is the second-most commonly affected organ
after the lungs. The most common lesions are erythema nodosum, plaques, maculopapular
eruptions, subcutaneous nodules, and lupus pernio. Treatment is not required, since the lesions
usually resolve spontaneously in 2–4 weeks. Although it may be disfiguring, cutaneous
sarcoidosis rarely causes major problems. Sarcoidosis of the scalp presents with diffuse or
patchy hair loss.
Heart:
Histologically, sarcoidosis of the heart is an active granulomatous inflammation surrounded by
reactive oedema. The distribution of affected areas is patchy with localised enlargement of
heart muscles. This causes scarring and remodelling of the heart, which leads to dilatation of
heart cavities and thinning of heart muscles. As the situation progresses, it leads to aneurysm
of heart chambers. When the distribution is diffuse, there would be dilatation of both ventricles
of the heart, causing heart failure and arrhythmia. When the conduction system in the
intraventricular septum is affected, it would lead to heart block, ventricular tachycardia and
ventricular arrhythmia, causing sudden death. Nevertheless, the involvement of pericardium
and heart valves are uncommon.

Blood:
Abnormal blood tests are frequent, accounting for over 50% of cases, but are not
diagnostic.Lymphopenia is the most common blood anomaly in sarcoidosis.Anemia occurs in
about 20% of people with sarcoidosis. Leukopenia is less common and occurs in even fewer
cases but is rarely severe. Thrombocytopenia and hemolytic anemia are fairly rare.
Nervous system:
Any of the components of the nervous system can be involved. Sarcoidosis affecting the
nervous system is known as neurosarcoidosis.Cranial nerves are most commonly affected,
accounting for about 5–30% of neurosarcoidosis cases, and peripheral facial nerve palsy, often
bilateral, is the most common neurological manifestation of sarcoidosis. It occurs suddenly and
is usually transient. The central nervous system involvement is present in 10–25% of
sarcoidosis cases. Other common manifestations of neurosarcoidosis include optic nerve
dysfunction, papilledema, palate dysfunction, neuroendocrine changes, hearing abnormalities,
hypothalamic and pituitary abnormalities, chronic meningitis, and peripheral neuropathy.
Bone, muscles and joints:
Sarcoidosis can be involved with the joints, bones, and muscles. This causes a wide variety of
musculoskeletal complaints that act through different mechanisms. About 5–15% of cases
affect the bones, joints, or muscles.
Arthritic syndromes can be categorized as acute or chronic. Sarcoidosis patients with acute
arthritis often also have bilateral hilar lymphadenopathy and erythema nodosum. These three
associated syndromes often occur together in Löfgren syndrome. The arthritis symptoms of
Löfgren syndrome occur most frequently in the ankles, followed by the knees, wrists, elbows,
38
and metacarpophalangeal joints. Usually, true arthritis is not present, but instead, periarthritis
appears as a swelling in the soft tissue around the joints that can be seen by ultrasonographic
methods.
Exocrine and endocrine:
Prolactin is frequently increased in sarcoidosis, between 3 and 32% of cases have
hyperprolactinemia this frequently leads to amenorrhea, galactorrhea, or nonpuerperal mastitis
in women. It also frequently causes an increase in 1,25-dihydroxy vitamin D, the active
metabolite of vitamin D, which is usually hydroxylated within the kidney, but in sarcoidosis
patients, hydroxylation of vitamin D can occur outside the kidneys, namely inside the immune
cells found in the granulomas the condition produces. 1, 25-dihydroxy vitamin D is the main
cause for hypercalcemia in sarcoidosis and is overproduced by sarcoid granulomata. Gamma-
interferon produced by activated lymphocytes and macrophages plays a major role in the
synthesis of 1 alpha, 25(OH) 2D3.

Parotid enlargement occurs in about 5–10% of cases. Bilateral involvement is the rule. The
gland is usually not tender, but firm and smooth. Dry mouth can occur; other exocrine glands
are affected only rarely. The eyes, their glands, or the parotid glands are affected in 20–50% of
cases.

Fig.14. Representation of effects of sarcoidosis

39
DIAGNOSIS
Diagnosis of sarcoidosis is a matter of exclusion, as there is no specific test for the condition.
To exclude sarcoidosis in a case presenting with pulmonary symptoms might involve a chest
radiograph, CT scan of chest, PET scan, CT-guided biopsy, mediastinoscopy, open lung
biopsy, bronchoscopy with biopsy, endobronchial ultrasound, and endoscopic ultrasound with
fine-needle aspiration of mediastinal lymph nodes (EBUS FNA). Tissue from biopsy of lymph
nodes is subjected to both flow cytometry to rule out cancer and special stains (acid fast bacilli
stain and Gömöri methenamine silver stain) to rule out microorganisms and fungi.
Serum markers of sarcoidosis, include: serum amyloid A, soluble interleukin-2 receptor,
lysozyme, angiotensin converting enzyme, and the glycoprotein KL-6.Angiotensin-converting
enzyme blood levels are used in the monitoring of sarcoidosis. A bronchoalveolar lavage can
show an elevated (of at least 3.5) CD4/CD8 T cell ratio, which is indicative (but not proof)
pulmonary sarcoidosis. In at least one study the induced sputum ratio of CD4/CD8 and level
of TNF was correlated to those in the lavage fluid.A sarcoidosis-like lung disease called
granulomatous–lymphocytic interstitial lung disease can be seen in patients with common
variable immunodeficiency (CVID) and therefore serum antibody levels should be measured
to exclude CVID.
Chest radiograph changes are divided into four stages:
• Bihilar lymphadenopathy
• Bihilar lymphadenopathy and reticulonodular infiltrates
• Bilateral pulmonary infiltrates
• Fibrocystic sarcoidosis typically with upward hilar retraction, cystic and bullous
changes
Cardiac magnetic resonance imaging (CMR) is one modality for diagnosing cardiac
sarcoidosis. It has 78% specificity in diagnosing cardiac sarcoidosis. Its T2-weighted imaging
can detect acute inflammation. Meanwhile, late gadolinium contrast (LGE) can detect fibrosis
or scar. Lesions at the subpericardium and midwall enhancement of basal septum or
inferolateral wall is strongly suggestive of sarcoidosis. MRI can also follow up on the treatment
efficacy of corticosteroids and prognosis of cardiac sarcoidosis.
PET scan is able to quantify disease activity which cannot be performed by CMR

40
 Fig.15.Hilar adenopathy especially on the person's left (AP CXR)

Fig.16.Hilar adenopathy especially on the person's left (lateral CXR)

Fig.1. Hilar adenopathy especially on the person's left (coronal CT)

Fig.18.Hilar adenopathy especially on the person's left (transverse CT)

41
DIFFERENTIAL DIAGNOSIS
Differential diagnosis includes metastatic disease, lymphoma, septic emboli, rheumatoid
nodules, granulomatosis with polyangiitis, varicella infection, tuberculosis, and atypical
infections, such as Mycobacterium avium complex, cytomegalovirus, and
cryptococcus.Sarcoidosis is confused most commonly with neoplastic diseases, such as
lymphoma, or with disorders characterized also by a mononuclear cell granulomatous
inflammatory process, such as the mycobacterial and fungal disorders.

TREATMENT
Treatments for sarcoidosis vary greatly depending on the patient. At least half of patients
require no systemic therapy.Most people (>75%) only require symptomatic treatment with
nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or aspirin. For those presenting
with lung symptoms, unless the respiratory impairment is devastating, active pulmonary
sarcoidosis is observed usually without therapy for two to three months; if the inflammation
does not subside spontaneously, therapy is institute.
Major categories of drug interventions include glucocorticoids, antimetabolites, biologic
agents especially monoclonal anti-tumor necrosis factor antibodies. Investigational treatments
include specific antibiotic combinations and mesenchymal stem cells. If drug intervention is
indicated, a step-wise approach is often used to explore alternatives in order of increasing side
effects and to monitor potentially toxic effects.
Corticosteroids, most commonly prednisone or prednisolone, have been the standard treatment
for many years. In some people, this treatment can slow or reverse the course of the disease,
but other people do not respond to steroid therapy. The use of corticosteroids in mild disease
is controversial because in many cases the disease remits spontaneously.
Antimetabolites:
Antimetabolites, also categorized as steroid-sparing agents, such as azathioprine, methotrexate,
mycophenolic acid, and leflunomide are often used as alternatives to corticosteroids.Of these,
methotrexate is most widely used and studied. Methotrexate is considered a first-line treatment
in neurosarcoidosis, often in conjunction with corticosteroids. Long-term treatment with
methotrexate is associated with liver damage in about 10% of people and hence may be a
significant concern in people with liver involvement and requires regular liver function test
monitoring. Methotrexate can also lead to pulmonary toxicity (lung damage), although this is
fairly uncommon and more commonly it can confound the leukopenia caused by sarcoidosis.
Due to these safety concerns it is often recommended that methotrexate is combined with folic
acid in order to prevent toxicity.
Immunosuppressants:
As the granulomas are caused by collections of immune system cells, particularly T cells, there
has been some success using immunosuppressants (like cyclophosphamide, cladribine,
chlorambucil, and cyclosporine), immunomodulatory (pentoxifylline and thalidomide), and
anti-tumor necrosis factor treatment (such as infliximab, etanercept, golimumab, and
adalimumab).In a clinical trial cyclosporine added to prednisone treatment failed to

42
demonstrate any significant benefit over prednisone alone in people with pulmonary
sarcoidosis, although there was evidence of increased toxicity from the addition of cyclosporine
to the steroid treatment including infections, malignancies (cancers), hypertension, and kidney
dysfunction. 27

43
 PNEUMOCONIOSIS
Pneumoconiosis is the general term for a class of interstitial lung diseases where inhalation of
dust has caused interstitial fibrosis. The three most common types are asbestosis, silicosis, and
coal miner's lung. Pneumoconiosis often causes restrictive impairment, although diagnosable
pneumoconiosis can occur without measurable impairment of lung function. Depending on
extent and severity, it may cause death within months or years, or it may never produce
symptoms. It is usually an occupational lung disease, typically from years of dust exposure
during work in mining; textile milling; shipbuilding, ship repairing, and/or shipbreaking;
sandblasting; industrial tasks; rock drilling (subways or building pilings); or agriculture. It is
one of the most common occupational diseases in the world 28.
The site of damage within the lung is a function of both the size and the toxicity of the inhaled
agent. Generally, particles with a median diameter of 0.5–10 μm can penetrate into the alveoli,
and those that are toxic to host cells (particularly macrophages) can cause permanent harm.
Many mechanisms are likely to be particle specific; in general, however, release of pro-
inflammatory cytokines (initially from alveolar macrophages) causes fibroblast formation and
eventual fibrosis. The propensity of different types of particles to cause fibrosis varies widely;
silica dust is highly fibrogenic, whereas iron dust is not. Coal-worker's pneumoconiosis (CWP)
is directly due to the effects of both coal and stone dust, and may be simple or complicated.
Often regarded as a historic disease, new cases continue to occur. Most cases do not progress
beyond the simple form, in which radiographic abnormalities are not associated with significant
symptoms or reduction in lung function. Complicated CWP is caused by progression to
fibrosis, and in severe cases confluent fibrotic masses are seen, predominantly in the upper
lobes. Silicosis, the pneumoconiosis caused by inhaled crystalline silica, also has both simple
and complex forms, and again new cases continue to arise.

Fig.19.Coal worker’s pneumoconiosis

44
EPIDEMIOLOGY
Over the past few decades, many measures have been put in place to protect workers against
dust inhalation. However, pneumoconiosis is still a threat to public health. According to the
Global Burden of Disease studies, although the worldwide prevalence of pneumoconiosis has
shown a downward trend since 2015, there are still a large number of patients. The prevalence
of pneumoconiosis is around 527,500 cases, with over 60,000 new patients reported globally
in 2017 .The mortality of pneumoconiosis patients remained at a high level in recent years,
with over 21,000 deaths each year since 2015.
Alarmingly, pneumoconiosis has re-emerged even in the United States and Australia, countries
with highly developed healthcare systems, high standards of workplace safety procedures, and
highly mechanized mining practices that reduce workers’ exposure to particles. It is possible
that other less developed countries, especially those with inadequate reporting systems, have
many patients that have not yet been diagnosed and reported.
ETIOLOGY
Pneumoconiosis results from the accumulation of fine inhaled particles that cause an
inflammatory reaction within the lung. Fibrotic pneumoconiosis predominates, and its cause
is the inhalation of particles like silica, asbestos fibers, beryllium, talc, and coal dust. Patient
history usually reflects long term exposure to noxious inhalants since dust induced interstitial
lung disease is latent. Exposure to these inhalants usually takes place in the workplace. The
length of employment correlates with the risk of pneumoconiosis.

PATHOPHYSIOLOGY
The reaction of the lung to mineral dusts depends on many variables, including size, shape,
solubility, and reactivity of the particles. For example, particles greater than 5 to 10 μm are
unlikely to reach distal airways, whereas particles smaller than 0.5 μm move into and out of
alveoli, often without substantial deposition and injury. Particles that are 1 to 5 μm in diameter
are the most dangerous, because they get lodged at the bifurcation of the distal airways. Coal
dust is relatively inert, and large amounts must be deposited in the lungs before lung disease is
clinically detectable. Silica, asbestos, and beryllium are more reactive than coal dust, resulting
in fibrotic reactions at lower concentrations. Most inhaled dust is entrapped in the mucus
blanket and rapidly removed from the lung by ciliary movement. However, some of the
particles become impacted at alveolar duct bifurcations, where macrophages accumulate and
engulf the trapped particulates. The pulmonary alveolar macrophage is a key cellular element
in the initiation and perpetuation of lung injury and fibrosis. Many particles activate the
inflammasome and induce IL-1 production. The more reactive particles trigger the
macrophages to release a number of products that mediate an inflammatory response and
initiate fibroblast proliferation and collagen deposition leading to Pneumoconiosis.
CLASSIFICATION
Depending upon the type of dust, the disease is given different names:
 Coal worker’s pneumoconiosis (also known as coal miner's lung, black lung or
anthracosis) – coal, carbon

45
  Aluminosis – Aluminium
 Asbestosis – asbestos
 Silicosis (also known as "grinder's disease" or Potter's rot) – crystalline silica dust
 Bauxite fibrosis – bauxite
 Berylliosis – beryllium
 Siderosis – iron
 Byssinosis – Byssinosis is caused by cotton dust inhalation and typically demonstrates
a different pattern of lung abnormalities than most other pneumoconiosis.
 Chalicosis – fine dust from stonecutting
 Silicosiderosis (also sometimes called iron miner's lung – mixed dust containing silica
and iron
 Labrador lung (found in miners in Labrador, Canada) – mixed dust containing iron,
silica and anthophyllite, a type of asbestos
 Stannosis – tin oxide
 Talcosis – talc
 Baritosis - a benign type of pneumoconiosis caused by barium inhalation; it typically
causes little or no overgrowth, hardening, and/or fibrosis.
 Mixed-dust pneumoconiosis

Fig.22. Mixed dust pneumoconiosis

SYMPTOMS
Pneumoconiosis can take a long time to develop, as dust can build up slowly or take many
years to cause a reaction in the lungs. This means that symptoms may not appear immediately
after dust particles have entered the lungs.
A person with pneumoconiosis may no longer work in an environment with dust that has caused
the disease.
The key symptoms of pneumoconiosis are:
-difficulty breathing, or shortness of breath
-a cough, which may produce phlegm
-tightness in the chest

46
These symptoms can be similar to those of a cold or chest infection. However, symptoms tend
to persist and could indicate pneumoconiosis if someone experiencing them has worked in an
environment with harmful dust particles.
If scarring in the lungs is severe, oxygen may be less able to make it into the bloodstream. Low
levels of oxygen in the blood can cause problems for other organs in the body, such as the heart
and brain.

RISK FACTORS
There are clear risk factors for pneumoconiosis and a range of jobs that are more likely to bring
people into contact with harmful dust.
Some examples of occupations that may bring workers into contact with dust particles that
cause pneumoconiosis include:
-plumbers, roofers, and builders who work with asbestos
-coal miners
-textile workers 29

DIAGNOSIS
A chest X-ray or CT scan can reveal inflammation, excess fluid, or scarring in the lungs. A test
may also be done to check how much oxygen is reaching the blood from the lungs. Sometimes
a biopsy may be needed to rule out other diseases.
The screening of pneumoconiosis primarily relies on observing a history of exposure to harmful
dusts.
While high-resloution computed tomography is more sensitive to the detection of early signs
than chest x-rays are.
Because imaging techniques cannot assess the functional status of the patient, pulmonary
function tests (PFT) are used as a supplementary method of the evaluation of the disease. PFT
can be used to assess dyspnea, distinguish between obstructive and restrictive diseases, and
assess disease severity. It is difficult to differentiate pneumoconiosis from similar diseases
because of its long incubation period and vague clinical symptoms. Diagnosis relies on
laboratory examinations, such as bronchoalveolar lavage fluid (BALF), to observe the sediment
in the alveoli and exclude similar pulmonary diseases.
Potential new diagnostic methods for pneumoconiosis:
Electrical impedance tomography (EIT) may be a potential method for the early diagnosis of
pneumoconiosis. The clinical symptoms of pneumoconiosis are difficult to observe during the
early stages of the disease. However, the electrical conductivity of the cells significantly
changes before clinical symptoms appear. EIT can accurately detect changes in electrical
conductivity to diagnose pneumoconiosis prior to the appearance of clinical symptoms.EIT is
relatively harmless and can be used for dynamic monitoring. This method is presently applied

47
in clinical settings to evaluate lung function. Once relevant diagnostic criteria are established,
this technology can immediately be used to diagnose pneumoconiosis in clinical practice.
However, because this method has no diagnostic specificity, it cannot replace the chest x-rays
as the diagnostic standard of pneumoconiosis.
Another diagnostic method is the three-dimensional magnetopneumography magnetic dipole
model (3D-MPG-MDM). Recent research has found that 3D-MPG-MDM can non-invasively
and inversely track the amount and distribution of particles within the lungs. These researchers
have found that 3D-MPG-MDM can diagnose pneumoconiosis caused by inhalation of metal
particles.This is a powerful and effective technique that can assess the risk of pneumoconiosis
by identifying how many particles are deposited in the lungs, which promotes earlier diagnosis
and subsequent intervention. Although the incidence of metal particle-related pneumoconiosis
could be effectively controlled using this novel technique, its safety, sensitivity, and specificity
have not yet been systematically evaluated. Much work is required before this method can be
applied in clinical settings.

Fig.21.Flow chart of diagnosis

MANAGEMENT
Despite the high incidence of pneumoconiosis over the past few decades, established clinical
treatments for pneumoconiosis are still very limited. The only life-saving therapeutic option
in end-stage pneumoconiosis is lung transplantation. Additionally, reports have demonstrated
that some clinical treatments may relieve symptoms and possibly improve life quality.The
treatment of pneumoconiosis includes integrated treatments and whole lung lavage. Integrated
treatments are primarily based on the patient’s common clinical symptoms, including cough,

48
chest pains, and shortness of breath. Furthermore, treating pneumoconiosis-related
complications (eg, respiratory infections, tuberculosis, chronic obstructive pulmonary disease,
and pneumothorax) and encouraging patients to perform rehabilitative exercises can improve
lung function and help relieve some symptoms.
Whole lung lavage is used to remove phlegm, secretion, and dust or fibrotic cytokines from the
patient’s airway to delay the progression of pneumoconiosis. This procedure typically works
best when used in the early stages of this disease,when most of the inhaled dust is still in the
pulmonary alveoli. However, so far, there is no evidence to support that whole lung lavage
exhibits beneficial effects on pulmonary function or lung fibrosis. As an invasive procedure, it
is not known whether whole lung lavage has long-term negative impacts on lung homeostasis.
Potential therapies for pneumoconiosis:
New drugs:
Currently, some drugs have been identified as having therapeutic effects on pneumoconiosis,
and the related agents are summarized in Table 1. Drugs used in the treatment of other diseases
may have the ability to treat pneumoconiosis. For example, the anti-fibrosis drug pirfenidone
used for idiopathic pulmonary fibrosis (IPF), the anti-inflammatory immune response drugs
hydroxychloroquin,corticosteroids, and infliximab, the antioxidant drug N-acetylcysteine, and
the vasodilators nicorandil and carvedilo may inhibit pulmonary inflammation or fibrosis in
pneumoconiosis experimental models. Corticosteroids with anti-inflammation properties have
been shown to relieve the clinical symptoms of patients with chronic beryllium disease 29.

Fig.22. Treatment for pneumoconiosis

49
 IDIOPATHIC PULMONARY FIBROSIS
Idiopathic pulmonary fibrosis (IPF), or fibrosing alveolitis, is a rare, progressive illness of
the respiratory system, characterized by the thickening and stiffening of lung tissue, associated
with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by
a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick
and stiff, which affects the tissue that surrounds the air sacs in the lungs 30.
The rate of progression can vary greatly from one person to another. Over years, most
individuals experience increasing respiratory symptoms, progressive scarring of the lungs and
a gradual decline in lung function. Less often, affected individuals have mild scarring within
the lungs and little to no change in the disease for many years. In some cases, the disorder can
progress rapidly (acutely), causing life-threatening complications within several years of
diagnosis.
The term ‘idiopathic’ means that the underlying cause of the disorder is unknown or unproven,
however, recently genetic susceptibility has been shown to account for 35-40% of the risk of
developing this disease. Although there is no cure for IPF, various different treatments are
available to manage the disorder and several newer therapeutic options are being studied.
Ultimately, some affected individuals will require a lung transplant.

EPIDEMIOLOGY
The incidence of IPF is difficult to determine as uniform diagnostic criteria have not been
applied consistently. A recent study from the USA estimated the incidence of IPF to be between
6.8 and 16.3 per 100,000 persons. In the 27 European Union countries, a range of sources
estimate an incidence of 4.6–7.4 people per 100,000 of the population, suggesting that
approximately 30,000–35,000 new patients will be diagnosed with IPF each year.
The prevalence of IPF has been estimated between 14.0 and 42.7 per 100,000 persons based
on a USA analysis of healthcare claims data, with variation depending on the case definitions
used in this analyses. IPF is more common in men than in women and is usually diagnosed in
people over 50 years of age.
ETIOLOGY
The cause of IPF is unknown but certain environmental factors and exposures have been shown
to increase the risk of getting IPF. Cigarette smoking is the best recognized and most accepted
risk factor for IPF, and increases the risk of IPF by about twofold. Other environmental and
occupation exposures such as exposure to metal dust, wood dust, coal dust, silica, stone dust,
biologic dusts coming from hay dust or mold spores or other agricultural products, and
occupations related to farming/livestock have also been shown to increase the risk for IPF.
There is some evidence that viral infections may be associated with idiopathic pulmonary
fibrosis and other fibrotic lung diseases.

50
PATHOPHYSIOLOGY
Despite extensive investigation, the cause of IPF remains unknown. The fibrosis in IPF has
been linked to cigarette smoking, environmental factors (e.g. occupational exposure to gases,
smoke, chemicals or dusts), other medical conditions including gastroesophageal reflux disease
(GERD), or to genetic predisposition (familial IPF). However, none of these is present in all
people with IPF and therefore do not provide a completely satisfactory explanation for the
disease.
IPF is believed to be the result of an aberrant wound healing process including/involving
abnormal and excessive deposition of collagen (fibrosis) in the pulmonary interstitium with
minimal associated inflammation. Cellular senescence is suspected to be a central contributing
cause, a belief which is supported by benefits seen in patients given senolytic therapy.

Fig.23.Pathophysiology
SIGNS AND SYMPTOMS
In many people, symptoms are present for a considerable time before diagnosis. The most
common clinical features of IPF include the following:
-Age over 50 years
-Dry, non-productive cough on exertion
-Progressive exertional dyspnea (shortness of breath with exercise)
-Dry, inspiratory bibasilar "velcro-like" crackles on auscultation (a crackling sound in the lungs
during inhalation similar to Velcro being torn apart slowly, heard with a stethoscope).
-Clubbing of the digits, a disfigurement of the finger tips or toes.
-Abnormal pulmonary function test results, with evidence of restriction and impaired gas
exchange.

51
Some of these features are due to chronic hypoxemia (oxygen deficiency in the blood), are not
specific for IPF, and can occur in other pulmonary disorders. IPF should be considered in all
patients with unexplained chronic exertional dyspnea who present with cough, inspiratory
bibasilar crackles, or finger clubbing.
Assessment of "velcro" crackles on lung auscultation is a practical way to improve the earlier
diagnosis of IPF. Fine crackles are easily recognized by clinicians and are characteristic of IPF.
If bilateral fine crackles are present throughout the inspiratory time and are persisting after
several deep breaths, and if remaining present on several occasions several weeks apart in a
subject aged ≥60 years, this should raise the suspicion of IPF and lead to consideration of an
HRCT scan of the chest which is more sensitive than a chest X-ray. As crackles are not specific
for IPF, they must prompt a thorough diagnostic process.
RISK FACTORS
Risk factors include cigarette smoking, acid reflux disease (GERD), certain viral infections,
and genetic predisposition.
DIAGNOSIS
A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published
by the American Thoracic Society (ATS) and the European Respiratory Society (ERS) in 2000
proposed specific major and minor criteria for establishing the diagnosis of IPF.However, in
2011, new simplified and updated criteria for the diagnosis and management of IPF were
published by the ATS, ERS, together with the Japanese Respiratory Society (JRS) and Latin
American Thoracic Association (ALAT).Currently, a diagnosis of IPF requires:
Exclusion of known causes of ILD, e.g., domestic and occupational environmental exposures,
connective tissue disorders, or drug exposure/toxicity
The presence of a typical radiological pattern of usual interstitial pneumonia (UIP) on high-
resolution computed tomography (HRCT).
In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating
the need for surgical lung biopsy 31.

RADIOLOGY
Chest X-rays are useful in the follow up routine of IPF patients. Plain chest X-rays are
unfortunately not diagnostic but may reveal decreased lung volumes, typically with prominent
reticular interstitial markings near the lung bases.
A chest radiograph of a patient with IPF. Note the small lung fields and peripheral pattern of
reticulonodular opacification.
The radiological evaluation through HRCT is an essential point in the diagnostic pathway in
IPF. HRCT is performed using a conventional computed axial tomographic scanner without
injection of contrast agents. Evaluation slices are very thin, 1–2 mm.

52
HRCT is an essential component of the diagnostic pathway in IPF which can identify UIP by
the presence of:
-Reticular opacities, often associated with traction bronchiectasis
-Honeycombing manifested as cluster cystic airspaces, typically of comparable diameters (3–
10 mm) but occasionally large.
-Ground-glass opacities are common but less extensive than the reticulation
-Distribution characteristically basal and peripheral though often patchy.

Fig.24. Small lung fields and peripheral pattern of reticulonodular opacification.

Fig.25.
High-resolution computed tomography scans of the chest of a patient with IPF.
The main features are of a peripheral, predominantly basal pattern of coarse reticulation with
honeycombing .

PULMONARY FUNCTION TEST

Spirometry classically reveals a reduction in the vital capacity (VC) with either a proportionate
reduction in airflows, or increased airflows for the observed vital capacity. The latter finding
reflects the increased lung stiffness (reduced lung compliance) associated with pulmonary
fibrosis, which leads to increased lung elastic recoil.
Measurement of static lung volumes using body plethysmography or other techniques typically
reveals reduced lung volumes (restriction). This reflects the difficulty encountered in inflating
the fibrotic lungs.

53
The diffusing capacity for carbon monoxide (DLCO) is invariably reduced in IPF and may be
the only abnormality in mild or early disease. Its impairment underlies the propensity of
patients with IPF to exhibit oxygen desaturation with exercise which can also be evaluated
using the 6-minute walk test (6MWT).
Terms such as 'mild', 'moderate', and 'severe' are sometimes used for staging disease and are
commonly based on resting pulmonary function test measurements. However, there is no clear
consensus regarding the staging of IPF patients and what are the best criteria and values to use.
Mild-to-moderate IPF has been characterized by the following functional criteria:
-Forced vital capacity (FVC) of ≥50%
-DLCO of ≥30%
-6MWT distance ≥150 meters .
TREATMENT
The goals of treatment in IPF are essentially to reduce the symptoms, stop disease progression,
prevent acute exacerbations, and prolong survival. Preventive care (e.g. vaccinations) and
symptom-based treatment should be started early in every patient.
Oxygen therapy
In the 2011 IPF guidelines, oxygen therapy, or supplementary oxygen for home use, became a
strong recommendation for use in those patients with significantly low oxygen levels at rest.
Although oxygen therapy has not been shown to improve survival in IPF, some data indicate
an improvement in exercise capacity.

Medications
A number of treatments have been investigated in the past for IPF, including interferon gamma-
1β, bosentan, ambrisentan, and anticoagulants, but these are no longer considered effective
treatment options. Many of these earlier studies were based on the hypothesis that IPF is an
inflammatory disorder.
Pirfenidone
A Cochrane review comparing pirfenidone with placebo, found a reduced risk of disease
progression by 30%. FVC or VC was also improved, even if a mild slowing in FVC decline
could be demonstrated only in one of the two CAPACITY trials. A third study, which was
completed in 2014 found reduced decline in lung function and IPF disease progression. The
data from the ASCEND study were also pooled with data from the two CAPACITY studies in
a pre-specified analysis which showed that pirfenidone reduced the risk of death by almost
50% over one year of treatment.
N-acetylcysteine and triple therapy
N-Acetylcysteine (NAC) is a precursor to glutathione, an antioxidant. It has been hypothesized
that treatment with high doses of NAC may repair an oxidant–antioxidant imbalance that
occurs in the lung tissue of patients with IPF. In the first clinical trial of 180 patients

54
(IFIGENIA), NAC was shown in previous study to reduce the decline in VC and DLCO over
12 months of follow-up when used in combination with prednisone and azathioprine (triple
therapy).More recently, a large randomized, controlled trial (PANTHER-IPF) was undertaken
by the National Institutes of Health (NIH) in the US to evaluate triple therapy and NAC
monotherapy in IPF patients. This study found that the combination of prednisone,
azathioprine, and NAC increased the risk of death and hospitalizationsand the NIH announced
in 2012 that the triple-therapy arm of the PANTHER-IPF study had been terminated early.
This study also evaluated NAC alone and the results for this arm of the study were published
in May 2014 in the New England Journal of Medicine, concluding that "as compared with
placebo, acetylcysteine offered no significant benefit with respect to the preservation of FVC
in patients with idiopathic pulmonary fibrosis with mild-to-moderate impairment in lung
function".
Nintedanib
Nintedanib is a triple angiokinase inhibitor that targets receptor tyrosine kinases involved in
the regulation of angiogenesis: fibroblast growth factor receptor (FGFR), platelet-derived
growth factor receptor (PDGFR), and vascular endothelial growth factor receptor (VEGFR),
which have also been implicated in the pathogenesis of fibrosis and IPF. In both phase III trials,
nintedanib reduced the decline in lung function by approximately 50% over one year. It was
approved by the US FDA in October 2014 and authorised in Europe in January 2015.
Lung transplantation
Lung transplantation may be suitable for those patients physically eligible to undergo a major
transplant operation. In IPF patients, lung transplant has been shown to reduce the risk of death
by 75% as compared with patients who remain on the waiting list. Since the introduction of the
lung allocation score (LAS), which prioritizes transplant candidates based on survival
probability, IPF has become the most common indication for lung transplantation in the USA.
Symptomatic patients with IPF younger than 65 years of age and with a body mass index (BMI)
≤26 kg/m2 should be referred for lung transplantation, but there are no clear data to guide the
precise timing for LTx. Although controversial, the most recent data suggest that bilateral lung
transplantation is superior to single lung transplantation in patients with IPF. Five-year survival
rates after lung transplantation in IPF are estimated at between 50 and 56%.
Palliative care
Palliative care focuses on reducing symptoms and improving the comfort of patients rather than
treating the disease. This may include treatment of worsening symptoms with the use of chronic
opioids for severe dyspnea and cough. Further, oxygen therapy may be useful for palliation of
dyspnea in hypoxemic patients. Palliative care also includes relief of physical and emotional
suffering and psychosocial support for patients and caregivers.With disease progression,
patients may experience fear, anxiety and depression and psychological counseling should
therefore be considered. In a recent study of outpatients with ILDs, including IPF, depression
score, functional status (as assessed by walk test), as well as pulmonary function, all
contributed to the severity of dyspnea.
In selected cases of particularly severe dyspnea morphine could be considered. It can reduce
dyspnea, anxiety and cough without significant decrease in oxygen saturation 31.

55
 PHYSIOTHERAPY MANAGEMENT

PULMONARY REHABILITATION
Pulmonary rehabilitation has been defined by the American Thoracic Society and European
Respiratory Society in 2013
Pulmonary rehabilitation (PR) is a “comprehensive intervention based on a thorough patient
assessment followed by patient-tailored therapies that include, but are not limited to, exercise
training, education, and behaviour change, designed to improve the physical and psychological
condition of people with chronic respiratory disease and to promote the long-term adherence
to health-enhancing behaviors”
PR (Pulmonary rehabilitation) is tailored to the individual who has recently had an
exacerbation, with the aim of optimizing their respiratory function and therefore their quality
of life (QOL) and participation in their everyday lives. PR (pulmonary rehabilitation) has been
proven to significantly improve health related QOL (quality of life) and exercise capacity in
individuals with Chronic Respiratory Disorders.
PR (pulmonary rehabilitation) programmes vary from person to person and from centre to
centre, depending on available resources, but in general will include:
-Multidisciplinary input, Exercise, Dietary advice, Disease education, Psychological
intervention, Behavioural intervention.
Typically, a session will involve a group of people with respiratory disorders attending a class
together at a gym or community hall where they partake in supervised exercise with a
physiotherapist. They then have an education session with an educator (typically a nurse or
allied health professional) on a range of topics, such as: bronchodilator technique; nutrition;
stress and anxiety; and managing the disease 32.

STRUCTURE
PR (pulmonary rehabilitation) programmes can vary in length, anywhere from 6-8 weeks to a
year. The British Thoracic Society’s guideline recommends 6-12 weeks with twice weekly
supervised exercise sessions (with a third unsupervised session), at a minimum of 12
supervised sessions. PR can be based in hospital, in the community or in both. Research
suggests that better outcomes are observed in inpatient-based PR compared to community-
based PR as measured by the Chronic Respiratory Questionnaire which measures dyspnoea,
fatigue, emotional function and mastery. Guidelines recommend that individuals be offered
some sort of exercise program after finishing PR (pulmonary rehabilitation).
ADHERENCE
NICE (National Institute for Health and Care Excellence) guidelines recommend emphasizing
the importance of adherence to PR (Pulmonary rehabilitation) to individuals in order to achieve
improvements in QOL (quality of life) and respiratory function. Specific ways to improve
adherence include optimising access to PR, this includes: suitable times for classes, appropriate

56
physical access to facilities, good public transport links and timely referrals to PR (pulmonary
rehabilitation).
MAINTENANCE
Guidelines recommend maintenance of exercise after PR to maintain gains made in respiratory
function, exercise tolerance and QOL. A recent study has shown that gains made after an eight-
week outpatient PR programme can be maintained at two years follow up in people with
moderate-to-severe Respiratory disorders with a maintenance programme. Individuals in this
study showed better maintenance in scores for 6-minute-walk distance and body mass index,
airflow obstruction, dyspnea score and exercise capacity. The maintenance programme was
focused on exercise and included cycle ergometers in homes and hospital-based supervised
exercise sessions every other week. Adherence to this maintenance programme was 66%.
CONTRAINDICATIONS
According to the NICE guideline, the following people should not undergo PR:
-Those who are unable to walk
-Those who have unstable angina
-People who have had a recent myocardial infarction
Other factors worth considering include:
-Unstable cardiac disease
-Locomotor issues
-Difficulties following instructions due to cognitive or psychiatric impairments
-The attendance of a support person to enable and encourage adherence 33.
INTERVENTION
Physiotherapists play an important role in prescribing, supervising, and measuring outcomes
in exercise. Research shows there has been an increase in the use of guideline-based exercise
prescription methodology (using FITT methodology - Frequency, Intensity, Time, Type).
Participants engaging in both functional and maximal exercise show statistically significant
improvements after PR, compared with usual care.
-Pulmonary care: Positioning, exercises, bronchiodialator, etc.
-Functional training: Important in end-stage disease or extreme weakness or fatigue
-Adapt the environment to improve ease
-Work areas supported in convenient places to avoid bending
-Locate a table to slide heavy objects while working
-Chairs at landings of stairs, besides bathtubs
-Using adaptive equipment and assistive technology
-Using good ventilation to kitchens etc. where fumes are present.

57
For mild lung disease:
-Have symptoms with extreme effort (cough, sputum)
-Spirometry: Show the predicted Vital Capacity (VC), FEV1 (Forced expiratory volume in the
1st second) is 70-80%
-ABG: normal, mild hypoxemia
-Exercise testing and training, individual exercises prescribed, formal rehab not required
For moderate lung disease:
-Subjects with moderate lung disease typically have shortness of breath on daily activities
-Episode of acute pneumonia after major surgery is when the pulmonary disease is identified,
demonstrates good results with PR
-VC and FEV1 55-70% (indicates shortness of breath at app 3-4 METS)
-Exercise testing: start at a low metabolic equivalent (MET) i.e. 1.5 MET and progress 0.5
MET at each stage, monitoring ECG, BP, and HR or perform a 12-minute walk test
Exercise prescription:
-Intensity: HR at a point when patient is 2-3 dyspnoeic on the Borgs scale of perceived exertion
-Frequency: 5-7 times a week
-If symptoms develop use O2
For Severe Lung disease:
-Shortness of breath on most activities of daily living
-VC and FEV1 <50%
-Needs oxygen at rest
-Some show R ventricular dysfunction
-Testing: low level intermittent test or exercises set at a steady endurance test of 2-3 METs
-Training: interval training, short exercise bouts, frequent rests, once a day, when duration
increases to 20 mins, 5 times a week
-Intensive monitoring:
When monitoring Oxygen, every decrease in SPO2 of 3%, should be noted. If the drop reached
<88% SPO2 oxygen therapy is indicated 33.

58
 Fig.26.Core components of pulmonary rehabilitation

CHEST PHYSIOTHERAPY
Chest physiotherapy is a broad term used in research that incorporates physiotherapy treatment
techniques that address the removal of secretion and improve airway clearance thereby help to
improve respiratory efficiency. Chest physiotherapy is the term for a group of treatments
designed to eliminate secretions, thus helping to decrease work of breathing, promote the
expansion of the lungs, and prevent the lungs from collapse 33.
The purpose of chest physiotherapy is:
-To facilitate removal of retained or profuse airway secretions.
-To optimize lung compliance and prevent it from collapsing.
-To decrease the work of breathing.
-To optimize the ventilation-perfusion ratio/ improve gas exchange.
CLASSIFICATION
There are various physiotherapy treatments incorporated within chest physiotherapy. Chest
physiotherapy techniques can be classified as conventional, modern, or instrumental techniques
based on evolving research.
Conventional Techniques:
Conventional chest physiotherapy is also known as traditional chest physiotherapy. It was
advocated first in 1915. It involves manual handling techniques to facilitate mucociliary
clearance. Postural drainage along with percussion and vibration (PDPV) was previously
widely named as Chest Physiotherapy. Later, coughing exercises and forced expiratory
techniques (huffing) were incorporated within it. PDPV with huffing has shown an effective
outcome. It can be self-administered or performed with the assistance of another person (a

59
physiotherapist, parent, or caregiver). PDPV works better if applied with bronchodilator
therapy 31.

POSTURAL DRAINAGE
Postural drainage involves positioning a person with the assistance of gravity to aid the normal
airway clearance mechanism. Postural drainage positioning varies based on specific segments
of the lungs with a large amount of secretions. Postural drainage is the drainage of secretions,
by the effect of gravity, from one or more lung segments to the central airways (where they can
be removed by a cough or mechanical aspiration). Each position consists of placing the target
lung segment(s) superior to the carina. Positions should generally be held for 3 to 15 minutes
(longer in special situations). Standard positions are modified as the patient's condition and
tolerance warrant. Before determining the postural drainage position, it is very important to
auscultate the lungs and identify the lung segments where added sound (Crepitus, Ronchi) is
heard. Postural drainage can be facilitated with percussion and vibration in the postural
drainage position.

Fig.27.Postural drainage positions

60
 Fig.28.Postions of postural drainage

PERCUSSION
Percussion is also referred to as cupping, clapping, and tapotement. The purpose of percussion
is to intermittently apply kinetic energy to the chest wall and lungs. This is accomplished by
rhythmically striking the thorax with a cupped hand or mechanical device directly over the
lung segment(s) being drained.

Fig.29.Percussion

61
VIBRATION
Vibration involves the application of a fine tremorous action (manually performed by pressing
in the direction that the ribs and soft tissue of the chest move during expiration) over the
draining area. In this technique, a rapid vibratory impulse is transmitted through the chest wall
from the flattened hands of the therapist by isometric alternate contraction of forearm flexor
and extensor muscles, to loosen and dislodge the airway secretions.

Fig.30.Vibration technique

Fig.31.Vibrator
COUGHING
Coughing includes directed coughing and various assisted coughing techniques.
FORCED EXPIRATORY TECHNIQUE
Forced expiratory techniques involve diaphragmatic inspiration, relaxing the scapulohumeral
region, and expiring forcefully from mid to low lung volumes whilst maintaining an open
glottis ("huffing" exercises). It is more effective than coughing.
INDICATIONS
Postural drainage positioning:

62
-Inability or reluctance of the patient to change body position. (e.g., mechanical ventilation, -
neuromuscular disease, drug-induced paralysis).
-Poor oxygenation associated with the position (eg, unilateral lung disease).
-Potential for or presence of atelectasis.
-Presence of artificial airway.
PDPV (POSTURAL DRAINAGE ALONG WITH PERCUSSION AND VIBRATION):
-Difficulty clearing secretions with expectorated sputum production greater than 25-30 mL/day
(adult).
-Evidence or suggestion of retained secretions in the presence of an artificial airway.
-Presence of atelectasis caused by or suspected of being caused by mucus plugging.
-Diagnosis of diseases such as cystic fibrosis, bronchiectasis, or cavitating lung disease
-Presence of foreign body in the airway.
-Patient with copious sputum or with central consolidation.
CONTRAINDICATIONS
Positioning:
All positions are contraindicated for:
-Intracranial pressure (ICP) > 20 mm Hg
-head and neck injury until stabilized (Absolute)
-Active hemorrhage with hemodynamic instability (Absolute)
-Recent spinal surgery (eg, laminectomy) or acute spinal injury
-Acute spinal injury or active hemoptysis
-Empyema
-Bronchopleural fistula
-Pulmonary edema associated with congestive heart failure
-Large pleural effusions
-Pulmonary embolism
-Aged, confused, or anxious patients who do not tolerate position changes
-Rib fracture, with or without flail chest
-Surgical wound or healing tissue
Trendelenburg position is contraindicated for:
-Intracranial pressure (ICP) > 20 mm Hg

63
-Patients in whom increased intracranial pressure is to be avoided (eg, neurosurgery,
aneurysms, eye surgery)
-Uncontrolled hypertension
-Distended abdomen
-Oesophageal surgery
-Recent gross hemoptysis related to recent lung carcinoma treated surgically or with radiation
therapy.
-Uncontrolled airway at risk for aspiration (tube feeding or recent meal)
-Reverse Trendelenburg is contraindicated in the presence of hypotension or vasoactive
medication.
-Recently placed transvenous pacemaker or subcutaneous pacemaker (particularly if
mechanical devices are to be used) 34.

COMPLICATIONS
-Hypoxemia
-Bronchospasm
-Increased Intracranial Pressure
-Acute Hypotension during Procedure
-Pulmonary Hemorrhage
-Pain or Injury to Muscles, Ribs, or Spine
-Vomiting and Aspiration
-Bronchospasm
-Dysrhythmias
FREQUENCY
Positioning:
Ventilated and critically ill patients: as necessary with the goal of once each hour or every other
hour as tolerated, around the clock. Less acute patients should be turned every 2 hours as
tolerated.
PDPV:
In critical care patients, including those on mechanical ventilation, PDT should be performed
every 4 to every 6 hours as indicated. PDT order should be re-evaluated at least every 48 hours
based on assessments from individual treatments.
In spontaneously breathing patients, frequency should be determined by assessing patient
response to therapy.
64
Acute care patient orders should be re-evaluated based on patient response to therapy at least
every 72 hours or with a change of patient status.
Domiciliary patients should be re-evaluated every 3 months and with a change of status.
MODERN TECHNIQUES
Over the years, several additional noninvasive clearance methods have been developed to
augment this traditional approach. Modern techniques use a variation of flow through breath
control to mobilize secretions. It includes an active cycle of breathing and autogenic drainage.
ACTIVE CYCLE OF BREATHING TECHNIQUE:
Active cycle of breathing technique (ACBT) is an active breathing technique performed by the
patient, and can be used to mobilize and clear excess pulmonary secretions and to generally
improve lung function. It has a series of three main phases: Breathing control, Thoracic
expansion, and Forced expiratory technique 34.

Fig.32.Active cycle of breathing techniques (ACBT)

Autogenic drainage
Autogenic drainage is a three‐phase active breathing technique using high expiratory flow rates
and variable lung volumes to unstick, collect, and evacuate secretions. The recipient is placed
sitting, back straight, and head slightly hyperextended, hands resting on the upper left and right
chest. The recipient first breathes at a low lung volume to unstick secretions in the peripheral
airways, then at mid‐volume to collect secretions in the central airways, and finally breathes at
high volume to clear secretions from the lungs. Autogenic drainage is potentially advantageous
because it improves independency. No equipment is needed, and it is applicable in different
settings and in daily life. The three phases of autogenic drainage are as follows.
Displacement: starts with a slow and forced oral expiration, recruiting a percentage of
expiratory reserve volume, and then carrying inspiration to low volume, recruiting percentages
of tidal volume followed by a two‐ to three‐second post-inspiratory pause. This is followed by
a slow oral exhalation recruiting a percentage of expiratory reserve volume.
Collection: nasal inspiration to medium volume, recruiting a larger percentage of tidal volume,
followed by a two‐ to three‐second post-inspiratory pause. This is followed by a slow oral
exhalation recruiting a percentage of expiratory reserve volume.

65
Elimination: nasal inspiration to high volume recruiting tidal volume and a percentage of
inspiratory reserve volume, followed by a two‐ to three‐second post-inspiratory pause, leading
to oral expiration at the level of tidal volume. The forced expiration technique is performed to
high volumes.

Fig.33.Autogenic drainage
INSTRUMENTAL TECHNIQUES:
Instrumental techniques such as non‐invasive ventilation have been considered useful as an
adjunct therapy to airway clearance and to provide respiratory support. Common instrumental
techniques are:
POSITIVE EXPIRATORY PRESSURE (PEP):
There are various positive expiratory pressure devices that provide resistance to expiration
through a mouthpiece or facemask, followed by forced expirations. The inhalation is at tidal
volume, and the expiration is slightly active against devices. These devices help to remove
secretions by increasing functional residual capacity and thus enhancing collateral ventilation
and removing secretions from collapse airways. Some of the devices are Flutter, Acapella, lung
flute, etc.

Fig.34.Postive expiratory pressure ( PEP)

66
CONTINUOUS POSITIVE AIRWAY PRESSURE (CPAP):
Continuous positive airway pressure (CPAP) is generated by exhalation against a constant
opening pressure; this produces positive end‐expiratory pressure (PEEP). Continuous positive
airway pressure can also be delivered by commercially available pressure drivers. These
generally require tightly fitting nasal prongs or a CPAP facemask. Bubble CPAP can be used
in a low resource environment and in the paediatric population. It consists of an interface (nasal
cannula), inspiratory tubing, and expiratory tubing immersed in an underwater bottle system.

Fig.35.Continuous positive air way pressure (CPAP)

HIGH FREQUENCY CHEST WALL OSCILLATION (HFCWO):
High-frequency chest wall oscillation (HFCWO) is an airway clearance technique in which
external chest wall oscillations are applied to the chest using an inflatable vest that wraps
around the chest. These machines produce vibrations at variable frequencies and intensities,
helping to loosen and thin mucus and separate it from airway walls.
HFCWO involves an inflatable jacket that is attached to a pulse generator by hoses that
mechanically enable the equipment to perform at variable frequencies (5–25 Hz). The generator
sends air through the hose, which causes the vest to inflate and deflate rapidly. The vibrations
not only separate mucus from the airway walls but also help move it up into the large airways.
Typically, it is paused during the 20- to 30-minute HFCWO treatment every 5 minutes to cough
out loosened mucus that has moved into the large airways.

Fig.36.High frequency chest wall oscillation (HFCWO)

67
INTRAPULMONARY PERCUSSIVE VENTILATION:
IPV was designed to promote mobilization of bronchial secretions and improve efficiency and
distribution of ventilation, providing intrathoracic percussion and vibration and an alternative
system for the delivery of the positive pressure to the lungs. Each IPV session lasted fifteen
minutes and was performed twice a day (morning and afternoon).Intrapulmonary percussive
ventilation uses a pneumatic device to deliver a series of pressurized gas minibursts at rates of
100 to 225 cycles per minute to the respiratory tract, by a mouthpiece. The duration of each
percussive cycle is manually controlled by a thumb button. During the cycle, constant PAP is
maintained at the airway. It also incorporates nebulizer for the delivery of the aerosol 35.

Fig.37.Intrapulmonary percussive ventilation

68
 SUMMARY
Restrictive lung diseases are a category of extra pulmonary, pleural, or parenchymal
respiratory diseases that restrict lung expansion, resulting in a decreased lung volume, an
increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary
function test demonstrates a decrease in the forced vital capacity.
Many forms of restrictive lung disease are progressive, getting worse over time. As the end of
life approaches, there may be changes that happen over days...or weeks. Each person's
experience is unique. People with end-stage lung disease often have significant airflow
limitations. Even simple tasks like taking a shower, making the bed and cooking a meal become
nearly impossible to do. In addition, people with end-stage lung disease often feel exhausted.
Typically, lung function drops to 30 percent or less during end-stage lung disease. Also, many
people have low blood oxygen levels.
Despite increasing mortality due to interstitial lung disease (ILD) the provision of end-of-life
care for these conditions is limited. Improving quality of care for patients with advanced ILD
or any other progressive restrictive lung disease requires a paradigm shift. Best supportive care
as it is currently experienced is far from best. Palliative care needs to sit alongside curative
intent as an equally legitimate professional goal.
Pulmonary rehabilitation is a comprehensive intervention based on a thorough patient
assessment followed by patient-tailored therapies, which include, but are not limited to,
exercise training, education, and Behavior change, designed to improve the physical and
psychological condition of people with chronic respiratory disease and to promote the long-
term adherence of health-enhancing behaviors.
The following are some of the problems faced by patients which physiotherapists can address:
Dyspnea, Altered lung function, Increased work of breathing and respiratory rate, Chest pain
& tightness, Mucus accumulation, Altered breathing mechanics, Altered quality of life, Fatigue
by exercises and therapies like postural drainage, percussion, vibration, forced expiratory
techniques, coughing, Active cycle of breathing technique (ACBT) and instrumental
techniques like Positive expiratory pressure (PEP),Continuous positive expiratory pressure
(CPAP), High frequency chest wall oscillation (HFCWO),Intrapulmonary percussive
ventilation(IPV).

69
 REFERENCES
1. Martinez-Pitre PJ, Sabbula BR, Cascella M. Restrictive lung disease.
2. Robinson HC. Respiratory Conditions Update: Restrictive Lung Disease. FP
essentials. 2016 Sep 1;448:29-34.
3. Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial
lung diseases. American journal of respiratory and critical care medicine. 1994
Oct;150(4):967-72.
4 Aggarwal AN, Agarwal R. The new ATS/ERS guidelines for assessing the
spirometric severity of restrictive lung disease differ from previous standards.
Respirology. 2007 Sep;12(5):759-62
5.. Mangera Z, Panesar G, Makker H. Practical approach to management of
respiratory complications in neurological disorders. International journal of general
medicine. 2012;5:255.
6.Fiorentino G, Esquinas AM. Restrictive lung disease: Low EPAP–Good ventilation.
Is it real?. Chronic Respiratory Disease. 2017 Aug;14(3):321-2.
7. . ANATOMY- B D CHAURASIA 6TH EDITION.
8.Hariri LP, Smith ML, Mino-Kenudson M, Allen TC, Attanoos R, Borczuk A, Burke L,
Cagle PT, Capelozzi V, Dacic S, Guinee D. Pulmonary pathology society perspective
on the 2018 American thoracic society, European respiratory society, Japanese
respiratory society, and Latin American thoracic society idiopathic pulmonary fibrosis
clinical practice guidelines. Annals of the American Thoracic Society. 2020
May;17(5):550-4.
9.Amaral AF, Coton S, Kato B, Tan WC, Studnicka M, Janson C, Gislason T, Mannino
D, Bateman ED, Buist S, Burney PG. Tuberculosis associates with both airflow
obstruction and low lung function: BOLD results. European Respiratory Journal. 2015
Oct 1;46(4):1104-12.
10. Bryant L. Merging the multi-measurement approach to Breathomics (Doctoral
dissertation, University of Leicester).
11 Kurth L, Hnizdo E. Change in prevalence of restrictive lung impairment in the US
population and associated risk factors: the National Health and Nutrition Examination
Survey (NHANES) 1988–1994 and 2007–2010. Multidisciplinary respiratory medicine.
2015 Dec;10(1):1-9.
12. Burke RR, Stone CH, Havstad S, Rybicki BA. Racial differences in sarcoidosis
granuloma density. Lung. 2009 Feb;187(1):1-7.
13. Mannino DM, McBurnie MA, Tan W, Kocabas A, Anto J, Vollmer WM, Buist AS,
BOLD Collaborative Research Group. Restricted spirometry in the burden of lung
disease study. The International journal of tuberculosis and lung disease. 2012 Oct
1;16(10):1405-11.

70
14. Lapinsky SE, Tram C, Mehta S, Maxwell CV. Restrictive lung disease in
pregnancy. Chest. 2014 Feb 1;145(2):394-8.
15. Nag N. Health state estimation. University of California, Irvine; 2020.
16.Lutfi MF. The physiological basis and clinical significance of lung volume
measurements. Multidisciplinary respiratory medicine. 2017 Dec;12(1):1-2.
17. Brinkman JE, Toro F, Sharma S. Physiology, respiratory drive. InStatPearls
[Internet] 2021 Aug 24. StatPearls Publishing.
18. Samardzic T, Mandiga P. Physiology, Pulmonary Stress Response. InStatPearls
[Internet] 2021 Sep 22. StatPearls Publishing.
19. Younger P. Elsevier's Dictionary of Medicine and Biology: In English, Greek,
German, Italian and Latin. Reference Reviews. 2006 Aug 1.
20. Case JK. Ferri's Differential Diagnosis: A Practical Guide to the Differential
Diagnosis of Symptoms, Signs, and Clinical Disorders. InMayo Clinic Proceedings
2006 Oct 1 (Vol. 81, No. 10, p. 1406). Elsevier.
21. Laveneziana P. Qualitative aspect of exertional dyspnea in patients with restrictive
lung disease. Multidisciplinary Respiratory Medicine. 2010 Dec;5(3):1-5.
22. Hutchinson J, Hubbard R, Raghu G. Surgical lung biopsy for interstitial lung
disease: when considered necessary, should these be done in larger and experienced
centres only?. European Respiratory Journal. 2019 Feb 1;53(2).
23. Johari J, Sharifudin MA, Ab Rahman A, Omar AS, Abdullah AT, Nor S, Lam WC,
Yusof MI. Relationship between pulmonary function and degree of spinal deformity,
location of apical vertebrae and age among adolescent idiopathic scoliosis patients.
Singapore medical journal. 2016 Jan;57(1):33.
24. Sulica R, Teirstein A, Padilla ML. Lung transplantation in interstitial lung disease.
Current Opinion in Pulmonary Medicine. 2001 Sep 1;7(5):314-21.
25.Taylor DR. Progressive respiratory disease: the importance of prognostic
conversations and advance care planning. Breathe. 2017 Dec 1;13(4):269-73.
26.Poletti V, Egan J. Classification, natural history and staging of idiopathic pulmonary
fibrosis. Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of
WASOG. 2013 Sep 1;30:13-20.
27 Baha A, Yıldırım F, Köktürk N, Galata Z, Akyürek N, Demirci NY, Türktaş H.
Cryptogenic and secondary organizing pneumonia: clinical presentation, radiological
and laboratory findings, treatment, and prognosis in 56 cases. Turkish thoracic journal.
2018 Oct;19(4):201.
28. Ardila-Gatas J, Sharma G, Nor Hanipah Z, Tu C, Brethauer SA, Aminian A, Tolle
L, Schauer PR. Bariatric surgery in patients with interstitial lung disease. Surgical
endoscopy. 2019 Jun;33(6):1952-8.

71
29. Qi XM, Luo Y, Song MY, Liu Y, Shu T, Liu Y, Pang JL, Wang J, Wang C.
Pneumoconiosis: current status and future prospects. Chinese Medical Journal. 2021
Apr 20;134(08):898-907.
30. .Naji NA, Connor MC, Donnelly SC, McDonnell TJ. Effectiveness of pulmonary
rehabilitation in restrictive lung disease. Journal of Cardiopulmonary Rehabilitation
and Prevention. 2006 Jul 1;26(4):237-43.
31. Bouros D, Nicholson AC, Polychronopoulos V, Du Bois RM. Acute interstitial
pneumonia. European Respiratory Journal. 2000 Feb 1;15(2):412-8.
32. Mavroudi M, Papakosta D, Kontakiotis T, Domvri K, Kalamaras G, Zarogoulidou
V, Zarogoulidis P, Latka P, Huang H, Hohenforst-Schmidt W, Zarogoulidis K. Sleep
disorders and health-related quality of life in patients with interstitial lung disease.
Sleep and Breathing. 2018 May;22(2):393-400.
33. Stoilova S, Stoilov M, Ramova E. Assessment of air pollution health risk from rek
bitola.
34. Selsby DS. Chest physiotherapy. BMJ: British Medical Journal. 1989 Mar
3;298(6673):541.
35. Sistach Bosch L. Development of a Virtual Reality Tool for Respiratory
Rehabilitation (Master's thesis, Universitat Politècnica de Catalunya).

72
 ANNEXURE
RESPIRATORY ASSESSMENT

Subjective Assessment:
Name:
Age:
Gender:
I.P No. / O.P No. :
Address
Occupation:
Date of Admission:
Date of assessment:
Department & Ward:
Chief Complaints:
Present History:
Past Diseases:
Associated illness: DM: YES /NO HTN: YES /NO TB: YES /NO
CAD: YES /NO COPD/ASTHMA: YES /NO
PERSONAL HISTORY –
Smoking –
Alcohol –
Drug Abuse-
Diet: - veg/ non veg/ mixed
Bowels: - Regular/ irregular/ constipated
Micturation: - normal/ abnormal
Habits/ Addiction:
Alcohol: - regular/ occasional/ retotaler
Tobacco: - snuff/ chewable/ smoking--- (pack/year)
Drugage: - yes/ no

73
Brettle nut: - yes/ no
Brettleleaf(pan): - yes/ no
MEDICAL HISTORY:–
a) PRESENT –
b) PAST –
PATIENTS MARITAL STATUS- (married / unmarried)
FAMILY HISTORY –
Family History of DM: YES /NO TB: YES /NO
HTN: YES /NO PSYCHIATRIC ILLNESS: YES /NO
STROKE: YES /NO SUDDEN CADIAC DEATH: YES /NO
ASTHMA: YES /NO HERDITARY DISEASE:
CANCER: YES /NO SIBLINGS History:
Any others:
SOCIOECONOMIC STATUS-
Other Diagnosis: Any orthopaedic problems: -
Any neurology problems: -
Any cardiology problems: -
Any integumentary problems: -
OBJECTIVE ASSESSMENT:
(I) On Observation (II) On Palpation (III) On Percussion
(IV) On Auscultation (V) Examination
(I) ON OBSERVATION
a. Evaluation of general appearance:
Level of consciousness: Alert/ Automatic/ Confused/ Delirious/ Stuporous/ Semi comatose/
Comatose
GCS Score:
Body type
Postural Abnormalities:
• Nicotin stains on naills & teeth Eg : heavy smoking
• Clubbing : Levibond angle [schmorth sign] 160 normal

74
• Cyanosis:
Central – lips, toung, tip of nose, mucus membrane.
Peripheral – toes, nails.
• Asterexis- flapping tremor of hands, when arms are extended due to hypercapnea
( co2)
Skin: Any incision:
Any Scar:
Any Trauma:
c) Evaluation of head & neck:
Face:
Neck:
d)Evaluation of Unmoving Chest:
Shape:
Symmetry:
Rib Angles:
Skin: scar/incision/trauma
Musculature around the chest:
e) Evaluation of moving chest:
Ventilatory Rate:
I:E ratio(insp&exp ratio):
Pattern of Breathing: abdominothoracic or thoracoabdominal breathing
F) Evaluation of speech:
• Dyspnea on phonation
• Poor voice volume control
G) Evaluation of Cough: Guidelines for evaluating cough:
Sputum: Amount: Colour: Consistency: Odour:
II) ON PALPATION:
Evaluation of chest symmetry
Chest wall pain:
Warmth:

75
Tenderness:
Tracheal position:
Vocal fremitus:
III) ON PERCUSSION:
Evaluation of diaphragmatic excursion:
IV) ON AUSCULTATION:
Breath sounds:
Vocal Resonance:
V) ON EXAMINATION:
Blood Pressure: Normal range:
Pulse Rate:
Respiratory rate:
Temperature:
Chest expansion measurement:
Axillary level -
Nipple level -
Xyphisternum level -
Exercise Capacity: Maximal O2 Uptake:
6 min walk/ 12 min walk test:
Shuttle walk test:
Borg’s rate of perceived exercises:
Laboratory Evaluation:
Chest X-ray:
ABG:
Spirometry:
CT/ MRI:
Pathology:
Microbiology:
• Differential Diagnosis:
Final Diagnosis:

76
Treatment
Problem list
Aims
Means
Goals
Short term
Long term
Home programme:
-Do’s
-Don’t’s

77