Chronic Cough Due to Bronchiectasis: ACCP

Evidence-Based Clinical Practice Guidelines

Mark J. Rosen

Chest 2006;129;122-131
DOI 10.1378/chest.129.1_suppl.122S
The online version of this article, along with updated information
and services can be found online on the World Wide Web at:
http://chestjournal.org/cgi/content/abstract/129/1_suppl/122S

CHEST is the official journal of the American College of Chest

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 Chronic Cough Due to Bronchiectasis
ACCP Evidence-Based Clinical Practice Guidelines
Mark J. Rosen, MD, FCCP

Background: Bronchiectasis is a condition that is characterized by the permanent dilation of

bronchi with destruction of elastic and muscular components of their walls, usually due to acute
or chronic infection. The cardinal symptom is a chronic productive cough.
Methods: Review of articles cited in the systematic literature search, along with others found in
Ovid MEDLINE and the Cochrane Library (including the Cochrane Database of Systematic
Reviews, the Cochrane Controlled Trial Register, and the Database of Abstracts of Reviews of
Effectiveness) from 1966 through 2003.
Results/conclusions: High-resolution CT scanning of the chest is the preferred means of
establishing the diagnosis of bronchiectasis. With the increasing use of antibiotics in the
treatment of childhood infection in the last several decades, an increasing percentage of
patients with bronchiectasis now have an underlying disorder that predisposes them to
chronic or recurrent infection. These include cystic fibrosis, common variable immunodefi-
ciency, HIV infection, primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis,
and chronic Mycobacterium avium complex infection. A variety of agents have been used to
improve cough effectiveness and prevent infectious exacerbations in patients with bronchi-
ectasis, with variable results. Chest physiotherapy offers a modest benefit in increasing
sputum volume, but its long-term effectiveness is unknown. Selected patients with localized
idiopathic bronchiectasis that causes intolerable symptoms despite maximal medical therapy
should be offered treatment with surgery. Patients with exacerbations of bronchiectasis
should be given antibiotics, with the choice of agents depending on the likely causative pathogens.
(CHEST 2006; 129:122S–131S)

Key words: allergic bronchopulmonary aspergillosis; antibiotics; bronchiectasis; bronchodilators; chest physiotherapy; cystic
fibrosis; mucolytics; Mycobacterium avium complex; primary ciliary dyskinesia

Abbreviations: ABPA ⫽ allergic bronchopulmonary aspergillosis; CF ⫽ cystic fibrosis; GERD ⫽ gastroesophageal reflux
disease; HRCT ⫽ high-resolution CT; MAC ⫽ Mycobacterium avium complex; PCD ⫽ primary ciliary dyskinesia;
rhDNase ⫽ recombinant human DNase

B ronchiectasis is a condition characterized by

the permanent dilation of bronchi with de-
in treating childhood infections, and with the
declining incidence of pulmonary tuberculosis.2– 4
struction of the elastic and muscular components In the last few decades, an increasing proportion
of their walls, usually due to acute or chronic of patients with bronchiectasis in developed coun-
infection.1,2 Bronchiectasis due to recurrent or tries have been found to have an underlying
severe infection in patients without an apparent disorder (disorders are summarized in Table 1).
underlying cause has become less common with Regardless of the etiology, the cardinal symptoms
the increasing use of immunization and antibiotics of bronchiectasis are chronic cough and sputum
production, although some patients may have a
Reproduction of this article is prohibited without written permission
from the American College of Chest Physicians (www.chestjournal. nonproductive cough. Recurrent bacterial coloni-
org/misc/reprints.shtml). zation and infection lead to progressive airway
Correspondence to: Mark J. Rosen, MD, FCCP, Division of injury that is mediated by neutrophils, T lympho-
Pulmonary and Critical Care Medicine, Beth Israel Medical
Center, First Ave and 16th St, New York, NY 11021; e-mail: cytes, and monocyte-derived cytokines.5,6 The ac-
mrosen@bethisraely.org tions of inflammatory mediators, elastase, and

122S Diagnosis and Management of Cough: ACCP Guidelines

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 Table 1—Disorders That Predispose the Patient to the Development of Bronchiectasis

Disorders Description

Focal distribution
Bronchial obstruction Foreign body
Tumor
Broncholithiasis
Compression by peribronchial lymph nodes
Previous pneumonia
Diffuse distribution
CF
Reduced host immunity Congenital and acquired hypogammaglobulinemia (especially IgG and/or IgG subclasses)
HIV infection
Primary ciliary dyskinesia
Allergic bronchopulmonary mycoses
Chronic MAC infection
Aspiration or toxic inhalation
Rheumatoid arthritis
Inflammatory bowel disease
Other congenital disorders ␣1-antitrypsin deficiency
Tracheobronchomegaly (Mounier-Kuhn syndrome)
Cartilage deficiency (Williams-Campbell syndrome)
Young syndrome
Pulmonary sequestration
Marfan syndrome
Yellow nail syndrome

collagenase lead in turn to inflammation and the Materials and Methods

destruction of the elastic and muscular compo-
The articles produced by the Duke systematic literature search
nents of bronchial walls, while the contractile were reviewed (see section 2 of this guideline), along with others
force of the surrounding lung tissue exerts trac- found in searches of Ovid MEDLINE and the Cochrane Library
tion, expanding the diameter of the involved air- (including the Cochrane Database of Systematic Reviews, the
ways.7,8 Bronchiectasis is also associated with in- Cochrane Controlled Trial Register, and the Database of Ab-
stracts of Reviews of Effectiveness) from 1966 through 2003.
creased bronchial arterial proliferation and
arteriovenous malformations, predisposing some
patients to recurrent hemoptysis.9
Diagnosis
Although bronchiectasis can be classified radio-
graphically and pathologically into cylindrical, var- Although chronic productive cough is the cardi-
icose, and cystic varieties,1 these distinctions are nal feature of bronchiectasis, this symptom occurs
not helpful in determining the etiology, treatment, far more commonly in patients with chronic bron-
and prognosis in individual patients.10 Bronchiec- chitis, asthma, upper airway cough syndrome due
tasis may have a focal or diffuse distribution.2 to a variety of rhinosinus diseases, previously
Focal bronchiectasis may follow a severe case of referred to as postnasal drip syndrome, and gas-
pneumonia, but with increasing use of antibiotics troesophageal reflux disease (GERD).11,12 In a
in recent years it is more often caused by bronchial prospective, before-and-after intervention trial,12
obstruction by a foreign body, broncholith, a a cohort of 71 immunocompetent adults who
slowly growing tumor, anatomic distortion follow- complained of expectoration of ⬎ 30 mL of spu-
ing lobectomy, or enlarged peribronchial lymph tum per day underwent a detailed protocol-driven
nodes. These predispose the patient to recurrent diagnostic evaluation; upper airway cough syn-
infection and bronchiectasis distal to the site of drome was the cause in 40% of patients, asthma in
obstruction. Focal bronchiectasis due to bronchial 24% of patients, GERD in 15% of patients, bron-
obstruction is important to recognize because it chitis in 11% of patients, and bronchiectasis in
may be amenable to local intervention with bron- only 4% of patients. Unusual causes of chronic
choscopic procedures or surgery. Diffuse bronchi- cough with excessive sputum production may be
ectasis is usually caused by an underlying disorder clinically unsuspected initially; these include non-
(Table 1). asthmatic eosinophilic bronchitis13 and bacterial

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suppurative airway disease.14 In the latter disor- firm the diagnosis. Level of evidence, low; benefit,
der, patients do not have excessive sputum expec- substantial; grade of recommendation, B
toration and may not have radiographic evidence
of bronchiectasis, but have copious purulent se-
cretions found on bronchoscopy. Specific Causes of Bronchiectasis
Excessive sputum volume, purulence, and tenacity
are nonspecific findings. The physical examination Many cases of bronchiectasis result from perma-
findings in patients with bronchiectasis may reveal nent bronchial wall damage after a childhood respi-
variable degrees of rhonchi, crackles, and clubbing, ratory infection, such as bacterial pneumonia, per-
or they may be normal. Also, the presence or tussis, or measles. In patients with healed
absence of crackles on auscultation of the chest does tuberculosis or other fibrosing lung diseases (eg,
not correlate with the presence of bronchiectasis as fungal infection, sarcoidosis, or idiopathic pulmonary
diagnosed by high-resolution CT (HRCT) scanning fibrosis), traction on major airways from surrounding
of the chest.4 Sputum cultures are often positive for fibrotic lung parenchyma may distort their architec-
Haemophilus influenzae, Staphylococcus aureus, ture, leading to recurrent infection and traction
Streptococcus pneumoniae, and Pseudomonas bronchiectasis.20 A systematic search for an underly-
aeruginosa, but these pathogens may also be found ing cause of bronchiectasis often yields a diagnosis
in the airways of patients with chronic bronchitis.15,16 that is amenable to treatment. In a study21 of 150
The isolation of mucoid strains of Pseudomonas adults with bronchiectasis, one or more causative
suggests the diagnosis of cystic fibrosis (CF), isola- factors were identified in 47% of cases, and the
tion of Aspergillus suggests allergic bronchopulmo- diagnosis had important prognostic or therapeutic
nary aspergillosis (ABPA), and isolation of Mycobac- significance in 15% of cases. In the latter category,
terium avium complex (MAC) suggests chronic patients with CF were referred to a specialty clinic,
infection with that organism, but isolating these patients with common variable immunodeficiency
organisms is not specific for these disorders. were started on Ig replacement therapy, patients
Because bronchiectasis is defined as abnormal with ABPA were given a trial of corticosteroid
dilation of airways, the diagnosis depends on visual- treatment, and a patient with recurrent aspiration
izing the typical changes either radiographically or associated with GERD was treated with an intensive
anatomically. Bronchiectasis is sometimes obvious regimen of gastric acid suppression.
on routine chest radiographs, but the diagnosis is
usually established using HRCT scanning.17,18 This
test compares favorably with bronchography, which Recommendation
had been considered a “gold standard,” but bron- 2. In patients for whom there is no obvious
chography is now rarely performed because it is cause, a diagnostic evaluation for an underlying
invasive, unpleasant, and risky, and because the disorder causing bronchiectasis should be per-
HRCT scan is as accurate in diagnosis, with sensitiv- formed, because the results may lead to treat-
ity and specificity exceeding 90%. ment that may slow or halt the progression of
The key feature of bronchiectasis on HRCT scans disease. Level of evidence, low; benefit, substantial;
is enlarged internal bronchial diameter, where the grade of recommendation, B
bronchi appear larger than the accompanying artery
(called signet ring sign).19 Other HRCT scan find-
ings in bronchiectasis include the failure of the larger CF
airways to taper while progressing to the lung pe-
riphery, air-fluid levels in dilated airways, and the Occurring in 1 per 2,000 to 3,000 live births, CF is
identification of airways in the extreme lung periph- probably the most common identifiable cause of
ery. Indirect HRCT scan signs of bronchiectasis bronchiectasis in the United States and Europe. A
include bronchial wall thickening, mucoid impac- mutation of the gene that encodes for the CF
tion, and focal air-trapping. transmembrane regulator protein disrupts the nor-
mal flow of fluid and electrolytes across cell mem-
branes, altering the composition of secretions in the
Recommendation respiratory tract, pancreas, GI tract, and sweat
glands.22 In the lung, this is manifested by excessive
1. In patients with suspected bronchiectasis and tenacious mucus secretion, impaired mucus
without a characteristic chest radiograph find- transport, dilation and hypertrophy of bronchial
ing, an HRCT scan should be ordered because glands, and goblet cell hyperplasia, leading to prox-
it is the diagnostic procedure of choice to con- imal airway plugging and bronchiectasis. This diag-

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nosis should be considered in children and younger (almost always in the dynein arms) and function.29
adults with recurrent sinopulmonary infection and/or Absent or discoordinated movement of cilia in the
bronchiectasis, even in the absence of GI symptoms. respiratory tract leads to recurrent sinus, ear, and
The sweat test (called pilocarpine iontophoresis) is lung infections, which in turn leads to bronchiectasis.
still the most accurate way to diagnose CF, as the The name Kartagener syndrome refers to the triad of
result is positive in almost all cases when performed situs inversus totalis, bronchiectasis, and either nasal
properly.23 Testing for the CF gene is less sensitive, polyps or recurrent sinusitis, occurring in approxi-
as there are ⬎ 700 mutations identified; commercial mately half of patients with primary ciliary dyskinesia
laboratories identify around 90% of CF mutations in (PCD).30 Male patients with PCD are almost always
the general population. infertile due to immotile sperm.29 The disorder is
diagnosed by assessing ciliary motility in semen
samples or in bronchial wall or nasal biopsy speci-
Reduced Host Immunity mens, or by electron microscopy that shows abnor-
mal cilia morphology. In a study29 of 78 patients with
Patients with congenital and acquired hypogam-
PCD diagnosed using a combination of compatible
maglobulinemia (especially involving IgG and/or IgG
clinical features and together with tests of ciliary
subclasses) have an increased frequency of recurrent
ultrastructure and function, chronic rhinitis/sinusitis
rhinosinusitis, respiratory infections, and bronchiec-
occurred in all patients, recurrent otitis media oc-
tasis.24 Hypogammaglobulinemia is diagnosed by
curred in 95% of patients, neonatal respiratory dis-
measuring the serum levels of IgG and IgG sub-
ease occurred in 73% of patients, and situs inversus
classes, along with those of IgM and IgA. In primary
occurred in 55% of patients. All patients had defects
hypogammaglobulinemias, the IgG level is typically
in the ciliary structure, two thirds of which occurred
⬍ 5 g/L, and the IgA level is ⬍ 0.1 g/L; antibody
in the outer dynein arm. Nasal nitric oxide produc-
deficiency can be confirmed by documenting de-
tion was very low compared with that in healthy
creased antibody production to two or more vac-
subjects and may be of diagnostic value. H influenzae
cines, including tetanus, diphtheria, measles,
was the most common bacterial isolate found in
mumps, and pneumococcal vaccine. Patients with
sputum, but Pseudomonas isolates were also com-
IgG deficiency may benefit from IV Ig replacement,
mon. Although the isolation of mucoid strains of
which is associated with a reduced frequency of
Pseudomonas in sputum is considered to be a
infection and a slowing of the progression of bron-
marker of CF, it was found in 15% of subjects with
chiectasis.25,26 Even though this therapy is consid-
PCD.
ered to be the standard of care for primary immu-
nodeficiency diseases with hypogammaglobulinemia,
there are no double-blind placebo-controlled trials
evaluating threshold Ig levels for starting treatment, ABPA
the effect of the standard dose of IV Ig (400 mg/kg
Immune-mediated inflammation in response to
every 28 days) on outcomes, and whether higher
inhaled Aspergillus fumigatus antigen causes this
doses are more effective.26 Those with isolated IgG
disorder in some patients with chronic asthma or
subclass deficiency and recurrent infection may ben-
CF.31 When a similar disorder is caused by other
efit from therapy with IV Ig, but this has not been
species of Aspergillus or other fungi, the term aller-
investigated in controlled clinical trials.26
gic bronchopulmonary mycosis may be preferable.32
Patients with HIV infection are also predisposed
The essential diagnostic criteria for the diagnosis of
to developing recurrent sinopulmonary infections
ABPA in patients with bronchiectasis are the central
and bronchiectasis, probably because of abnormal
distribution of disease (inner two thirds of chest on
B-lymphocyte function.27,28 The treatment of pa-
CT scan), asthma, immediate cutaneous reactivity to
tients with HIV infection and bronchiectasis with IV
Aspergillus antigen, elevated total serum IgE level
Ig has not been studied systematically. The impact of
(⬎ 417 IU/L or 1,000 ng/mL), and elevated serum
combination antiretroviral therapy on the severity
levels of IgG-A fumigatus (Aspergillus precipitins) or
and course of airway disease is also unknown, al-
IgE-A fumigatus in comparison to those seen in sera
though the restoration of immune function would be
from skin-test positive patients who did not have
expected to be beneficial.
ABPA.33 Patients may have pulmonary infiltrates and
eosinophilia, but these findings are not needed to
Primary Ciliary Dyskinesia establish the diagnosis. Mucoid impaction of the
bronchi and bronchocentric granulomatosis are also
This refers to a group of autosomal-recessive associated with ABPA.
disorders associated with defective ciliary structure As the disease is a manifestation of a hypersensi-

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tivity reaction rather than an infection, treatment is MAC, but the presence of granulomas in the airways
aimed at immune modulation. The administration of and the response to antimycobacterial therapy indi-
oral prednisone to patients with ABPA is associated cates that MAC infection is the primary disorder that
with the improvement of asthma, and the presence leads to progressive airway damage and bronchiec-
of pulmonary infiltrates and eosinophilia, with reduc- tasis. The diagnostic criteria for pulmonary infection
tion in serum levels of IgE, and probably reduced with MAC have been reviewed elsewhere and are
progression of bronchiectasis. The optimal dose is based on expert opinion.40 Sputum cultures are not
unknown, but 0.5 mg/kg/d is recommended, fol- sufficiently sensitive to establish the diagnosis in
lowed by a gradual taper and adjustment based on many patients.41 In patients with typical clinical and
the patient’s condition.34 The long-term use of cor- radiographic features of MAC infection, it is diag-
ticosteroids is often necessary, but carries risk, in- nosed in some patients with bronchoscopic biopsies,
cluding the development of invasive Aspergillus in- and other patients are treated empirically. There
fection. Therefore, the antifungal agent itraconazole have been no large randomized trials of the treat-
was tested in patients with ABPA as an adjunctive, ment of MAC-associated lung disease, but based on
steroid-sparing agent in two randomized placebo- in vitro data, there is expert consensus that treat-
controlled studies.35,36 Both studies spanned 16 ment with a macrolide (eg, clarithromycin or azithro-
weeks of treatment and showed reduced levels of mycin) with ethambutol and a rifamycin (eg, rifabu-
markers of systemic immune activation (serum IgE tin or rifampin) constitute first-line therapy for
level and eosinophil count). The study by Wark et patients with severe or progressive symptoms.40
al36 also showed reduced levels of markers of airway However, these regimens are prolonged and often
inflammation in induced sputum. Neither study35,36 poorly tolerated, and patients often relapse.
showed significant changes in lung function, al-
though the study by Wark et al36 showed that
subjects receiving itraconazole experienced fewer Other Disorders
exacerbations of disease requiring increased doses of
corticosteroids. Therefore, while itraconazole ap- Up to 3% of patients with rheumatoid arthritis
pears to be promising as adjunctive treatment for have symptomatic bronchiectasis, and up to 30%
patients with ABPA, long-term trials are needed to have HRCT-diagnosed disease.42 Some have recom-
assess the clinical efficacy and safety in patients in mended testing for serum rheumatoid factor in
different disease severity strata. There have been no patients with bronchiectasis with no defined etiology.
randomized controlled trials to evaluate the use of Inflammatory bowel disease, and especially chronic
antifungal therapies in patients with ABPA compli- ulcerative colitis, is associated with recurrent respi-
cating CF.37 ratory tract infection and bronchiectasis.43 Mecha-
nisms may include airway infiltration by immune
effector cells, enhanced autoimmunity, and compli-
MAC Infection cations of immune-modulating therapy.2 A variety of
congenital disorders listed in Table 1 predispose the
A clinicopathologic subtype of chronic pulmonary patient to chronic respiratory infection and bronchi-
infection with this pathogen occurs predominantly in ectasis; these have been discussed in detail else-
patients who are white women in their seventh or where.44
eighth decade of life with no underlying immune
compromise (called the Lady Windemere syn-
drome.)38,39 It is unknown why this group is at Treatment
increased risk, but an undefined inherited or ac-
quired immune deficiency state is presumed to exist. In addition to the treatment of an identified
These patients typically present with chronic cough, underlying disease, pharmacotherapy may be used
which is often associated with fever and weight loss. to enhance bronchodilation and improve mucocili-
The symptoms are insidious, and there is often an ary clearance, antibiotics may be used to prevent
interval of several years between the onset of illness and treat recurrent infection, maneuvers may be
and the diagnosis. Radiographs and CT scans show designed to mobilize secretions (eg, chest physio-
bronchiectasis and nodular densities, especially in therapy), and mucolytic agents and occasionally
the middle lobe and lingula.17 The nodules are surgery may be used to treat localized disease. The
typically distributed around peripheral vessels and goal of treatment is generally to improve the
airways, frequently with a tree-in-bud configuration. symptoms of cough, sputum production, and dys-
In the past, these patients were thought to have pnea, and to prevent the progression of airway
preexisting bronchiectasis and “colonization” with damage. Evaluating the effects of treatment in

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patients with bronchiectasis is limited by study Recommendation
methodology. Randomized trials of these treat-
ments generally lack proper control groups, and 4. In patients with bronchiectasis caused by
the surrogate end point of “effectiveness” is usu- CF, rhDNase should be used to improve spi-
ally the volume of sputum production or the rometry. Level of evidence, low; benefit, small;
clearance of radiolabeled aerosol from the lung. grade of recommendation, C
More meaningful studies should focus on the
number of exacerbations over time and on mea- Antiinflammatory Agents
sures of health-related quality of life. Inhaled corticosteroids are used commonly to
treat bronchial inflammation and to prevent exacer-
bations of asthma and COPD. Two randomized
Treatment of Stable Bronchiectasis short-time (4 and 6 weeks, respectively) placebo-
Bronchodilators controlled trials53,54 of inhaled corticosteroids in
patients with idiopathic bronchiectasis showed non-
Bronchodilators, including short-acting and long- significant trends toward improved FEV1, FVC,
acting ␤-agonists, anticholinergics, leukotriene an- diffusing capacity of the lung for carbon monoxide,
tagonists, and theophylline are useful in the manage- and residual volume, but no effect on toal lung
ment of COPD and/or asthma. Because many capacity, sputum production, cough, wheeze, or
patients with bronchiectasis also have COPD, and dyspnea. Oral corticosteroids may be more effective
many show signs of airflow obstruction and bronchial in treating bronchial inflammation, but there have
hyperreactivity, patients commonly receive broncho- been no randomized controlled trials addressing
dilator therapy. However, there have been no ran- clinical end points in patients with bronchiectasis,
domized studies that have validated their usefulness other than when it is associated with known cortico-
in the management of cough, sputum production, steroid-responsive conditions like asthma, COPD,
dyspnea, or other end points in patients with bron- rheumatoid arthritis, and ulcerative colitis.55 How-
chiectasis.45– 48 ever, the administration of oral corticosteroids at a
prednisolone-equivalent dose of 1 mg/kg on alter-
nate days appears to slow the progression of lung
Recommendation disease in CF patients, but is associated with signif-
icant side effects, especially in children.56 –59 There-
3. In patients with bronchiectasis with air- fore, systemic corticosteroid administration may be
flow obstruction and/or bronchial hyperreactiv- considered in patients with CF, but concerns about
ity, therapy with bronchodilators may be of side effects preclude a general recommendation for
benefit. Level of evidence, expert opinion; benefit, its use.
small; grade of recommendation, E/C

Mucolytics Recommendation
The purpose of mucolytic drugs is to assist 5. In patients with CF, prolonged treatment
tracheobronchial clearance by altering the proper- with systemic corticosteroids should not be of-
ties of sputum. Of this class of agents, only fered to most patients because of significant
recombinant human DNase (rhDNase) was stud- side effects. Level of evidence, low; benefit, con-
ied in randomized controlled trials in stable pa- flicting; grade of recommendation, I
tients with bronchiectasis not caused by CF.49 –51 Similarly, the administration of ibuprofen to pa-
DNA release by neutrophils in airways increases tients with CF with the goal of reducing inflamma-
sputum viscosity, and rhDNase administered by tion showed modest benefits in patients with mild
aerosol digests DNA, thereby decreasing sputum disease, but its use also cannot be recommended at
viscosity and hopefully mucus plugging, infection, this time because of the potential of side effects with
and inflammation. This agent was not associated prolonged use.60
with significant benefit in these two trials, and
cannot be recommended for patients with idio-
pathic bronchiectasis. However, patients with CF Recommendation
treated with rhDNase enjoyed spirometric im-
provement for up to 2 years compared with pla- 6. In patients with CF, prolonged courses of
cebo, with a nonsignificant reduction of risk of ibuprofen should not be used. Level of evidence,
infectious exacerbations.52 low; benefit, conflicting; grade of recommendation, I

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Antibiotics Bronchopulmonary Hygiene
The systemic and inhaled administrations of A variety of manual and mechanical interventions,
antibiotics were investigated as ways to prevent including chest percussion, vibration, postural drain-
exacerbations of bronchiectasis by breaking the age, mechanically assisted cough, and airway oscilla-
putative vicious cycle of bacterial colonization tion, are used commonly to facilitate the expectora-
leading to inflammation and further airway injury. tion of mucus. These maneuvers are also referred to
A literature review by the Cochrane group re- as chest physiotherapy, and formerly as pulmonary
vealed six randomized controlled trials61 in pa- toilet. Their primary role is to facilitate the clearance
tients with idiopathic bronchiectasis, but these of lower airway secretions in conditions associated
studies had differences in design that limited the with the hypersecretion of mucus and the inability to
ability to generalize the outcomes. In general, they expectorate effectively, as in patients with bronchi-
suggested that long-term therapy with antibiotics ectasis. The role of bronchopulmonary hygiene in
is effective for reducing sputum volume and pu- treating bronchiectasis and chronic bronchitis is
rulence, but has limited impact on the frequency discussed in a later section of this guideline. Al-
of exacerbations and the natural history of the though these techniques are considered to be main-
condition, and may facilitate the emergence of stays in the treatment of bronchiectasis, they have
resistant organisms. modest effects on increasing sputum volume, and
the long-term effectiveness of these interventions is
unknown.
Recommendation
7. In patients with idiopathic bronchiectasis, Recommendation
the prolonged systemic administration of anti-
10. In patients with conditions associated
biotics may produce small benefits in reducing
with the hypersecretion of mucus and the in-
sputum volume and purulence, but may also be
ability to expectorate effectively, chest physio-
associated with intolerable side effects. Level of
therapy should be used and patients should be
evidence, low; benefit, conflicting, grade of recom-
monitored for symptom improvement. Level of
mendation, I
evidence, expert opinion; benefit, small/weak; grade
A randomized placebo-controlled trial62 of inhaled
of recommendation, E/C
tobramycin delivered by jet nebulizer in patients
with CF showed that it was well tolerated, and was Surgery
associated with improved pulmonary function, de-
creased density of P aeruginosa in sputum, and There have been no randomized controlled stud-
decreased risk of hospitalization. ies comparing surgery with more conservative man-
agement, and the literature consists of retrospective
reviews of case series. With proper patient selection,
Recommendation surgery is associated with excellent outcomes and
low perioperative mortality rates.64,65 Surgery to
8. In patients with CF, therapy with aerosol- resect the bronchiectatic lung should be limited to
ized antipseudomonal antibiotics are recom- patients with local disease who have not responded
mended. Level of evidence, low; benefit, interme- to medical therapy. Double-lung transplantation is
diate; grade of recommendation, C commonly offered to patients with CF.
However, a similar trial63 in patients with bronchi-
ectasis not caused by CF was less encouraging;
although the treatment group had decreased density Recommendation
of P aeruginosa and was judged more likely to have
11. In selected patients with localized bron-
an “improved medical condition,” they were also
chiectasis that causes intolerable symptoms de-
more likely to report increased cough, dyspnea,
spite maximal medical therapy, surgery should
wheezing, and noncardiac chest pain.
be offered. Level of evidence, low; benefit, substan-
tial; grade of recommendation, B
Recommendation
Treatment of Exacerbations
9. In patients with idiopathic bronchiectasis,
aerosolized antibiotics should not be used. Level As bacterial exacerbations of bronchiectasis account
of evidence, low; benefit, negative; recommendation, D for substantial morbidity, antibiotics are a mainstay of

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treatment. Antibiotics are probably effective for exac-
erbations characterized by change in sputum produc- Summary of Recommendations
tion, increased dyspnea, increased cough, fever, in- 1. In patients with suspected bronchiec-
creased wheezing, malaise, fatigue, lethargy, reduced tasis without a characteristic chest radio-
exercise tolerance, reduced pulmonary function, new graph finding, an HRCT scan should be
pulmonary opacities (although this may represent ordered because it is the diagnostic proce-
pneumonia), and changes in chest sounds.51 dure of choice to confirm the diagnosis.
Level of evidence, low; benefit, substantial;
grade of recommendation, B
2. In patients for whom there is no obvious
Recommendation cause, a diagnostic evaluation for an underly-
12. In patients with exacerbations of bronchi- ing disorder causing bronchiectasis should be
ectasis, antibiotics should be used, with the performed, because the results may lead to
selection of agents depending on the likely treatment that may slow or halt the progres-
pathogens. Level of evidence, low; benefit, substan- sion of disease. Level of evidence, low; benefit,
tial; grade of recommendation, B substantial; grade of recommendation, B
3. In patients with bronchiectasis with air-
There have been no published randomized place-
flow obstruction and/or bronchial hyperreac-
bo-controlled trials on the antibiotic treatment of
tivity, therapy with bronchodilators may be of
exacerbations of bronchiectasis since the last Amer-
benefit. Level of evidence, expert opinion; bene-
ican College of Chest Physicians cough guideline,66 fit, small; grade of recommendation, E/C
and no new recommendations can be offered. Treat- 4. In patients with bronchiectasis caused
ment should initially include an agent that is active by CF, rhDNase should be used to improve
against H influenzae and S aureus; patients with spirometry. Level of evidence, low; benefit,
recent antibiotic exposure or CF should be consid- small; grade of recommendation, C
ered to be at high risk of infection with Pseudomonas 5. In patients with CF, prolonged treat-
and treated accordingly. Patients should receive at ment with systemic corticosteroids should
least 2 weeks of antimicrobial therapy; sputum cul- not be offered to most patients because of
ture and sensitivity testing should be performed in significant side effects. Level of evidence,
patients who do not have an adequate response, and low; benefit, conflicting; grade of recommen-
they should guide the choice of a longer course of an dation, I
oral agent, or even prolonged IV antibiotic therapy.2 6. In patients with CF, prolonged courses
of ibuprofen should not be used. Level of
evidence, low; benefit, conflicting; grade of rec-
Conclusions ommendation, I
7. In patients with idiopathic bronchi-
Bronchiectasis is diagnosed in approximately 4% ectasis, the prolonged systemic adminis-
of patients with chronic cough. HRCT scanning of tration of antibiotics may produce small
the chest is the best test to establish this diagnosis. benefits in reducing sputum volume and
Most cases of bronchiectasis in adults are idiopathic, purulence, but may also be associated
but in the absence of an obvious cause, a diagnostic with intolerable side effects. Level of evi-
evaluation for an underlying disorder should be dence, low; benefit, conflicting, grade of rec-
performed, as treatment may slow or halt the pro- ommendation, I
gression of airway disease. Bronchodilator agents, 8. In patients with CF, therapy with aero-
anticholinergic agents, and oral and inhaled steroids solized antipseudomonal antibiotics are rec-
are often used in stable patients with bronchiectasis ommended. Level of evidence, low; benefit,
as well as during acute exacerbations despite the lack intermediate; grade of recommendation, C
of objective evidence of benefit. Antibiotics and 9. In patients with idiopathic bronchiec-
bronchopulmonary hygiene are mainstays in the tasis, aerosolized antibiotics should not be
treatment of exacerbations, but the prolonged use of used. Level of evidence, low; benefit, negative;
systemic and inhaled antibiotics cannot be recom- recommendation, D
10. In patients with conditions associated
mended to prevent these episodes except in patients
with the hypersecretion of mucus and the
with bronchiectasis caused by CF. Surgery is bene-
inability to expectorate effectively, chest
ficial in a carefully selected subgroup of patients with
physiotherapy should be used and patients
focal disease and intractable symptoms.

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Chronic Cough Due to Bronchiectasis: ACCP Evidence-Based Clinical
Practice Guidelines
Mark J. Rosen
Chest 2006;129;122-131
DOI 10.1378/chest.129.1_suppl.122S
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