This document provides an overview of different types of inflammatory arthritis, including Juvenile Idiopathic Arthritis (JIA), Felty's Syndrome, Palindromic Rheumatism, and Sjogren's Syndrome. It describes the characteristic symptoms, physical exam findings, diagnostic criteria and complications associated with each condition. Key information includes that JIA is the most common chronic pediatric rheumatologic disease, Felty's Syndrome involves a triad of rheumatoid arthritis, splenomegaly, and granulocytopenia, and Sjogren's Syndrome features lymphocytic infiltration of lacrimal and salivary glands leading to dry eyes and mouth.
This document provides an overview of different types of inflammatory arthritis, including Juvenile Idiopathic Arthritis (JIA), Felty's Syndrome, Palindromic Rheumatism, and Sjogren's Syndrome. It describes the characteristic symptoms, physical exam findings, diagnostic criteria and complications associated with each condition. Key information includes that JIA is the most common chronic pediatric rheumatologic disease, Felty's Syndrome involves a triad of rheumatoid arthritis, splenomegaly, and granulocytopenia, and Sjogren's Syndrome features lymphocytic infiltration of lacrimal and salivary glands leading to dry eyes and mouth.
This document provides an overview of different types of inflammatory arthritis, including Juvenile Idiopathic Arthritis (JIA), Felty's Syndrome, Palindromic Rheumatism, and Sjogren's Syndrome. It describes the characteristic symptoms, physical exam findings, diagnostic criteria and complications associated with each condition. Key information includes that JIA is the most common chronic pediatric rheumatologic disease, Felty's Syndrome involves a triad of rheumatoid arthritis, splenomegaly, and granulocytopenia, and Sjogren's Syndrome features lymphocytic infiltration of lacrimal and salivary glands leading to dry eyes and mouth.
This document provides an overview of different types of inflammatory arthritis, including Juvenile Idiopathic Arthritis (JIA), Felty's Syndrome, Palindromic Rheumatism, and Sjogren's Syndrome. It describes the characteristic symptoms, physical exam findings, diagnostic criteria and complications associated with each condition. Key information includes that JIA is the most common chronic pediatric rheumatologic disease, Felty's Syndrome involves a triad of rheumatoid arthritis, splenomegaly, and granulocytopenia, and Sjogren's Syndrome features lymphocytic infiltration of lacrimal and salivary glands leading to dry eyes and mouth.
Nail involvement - pitting is the most common but
least specific finding
Disordered bone growth with resultant shortening
Sacroiliitis Enthesis related arthritis
Paediatric spondyloarthropathy
Inflammation of enthesis
Pain,tenderness and swelling at enthesis
Mainly the finger joints
Sacroilitis only in the later stage
Diagnostic considerations
Arthritis and enthesitis co-exist
Presence of arthritis +/ or enthesitis with two of the five below Sacroiliac tenderness and/or inflammatory lumbosacral pain Positive human leukocyte antigen B27 (HLA-B27) test continued
Onset of arthritis in a male 6 years old or older
Acute symptomatic anterior uveitis
Presence in a first-degree relative of ankylosing
spondylitis, enthesitis-related arthritis, inflammatory bowel disease with sacroiliitis, reactive arthritis, or acute anterior uveitis Undifferentiated arthritis
If the symptoms NOT fitting into any
groups OR
If it fits into more than one group
Investigations
No investigation is confirmatory
JIA is a clinical diagnosis
ESR and CRP may be elevated
Thrombocytosis, leucocytosis etc can be seen
ANA + points to uveitis
Complications
Macrophage Activating Syndrome
Cytokine storm is the pathogenic process
behind Suspect MAS if Falling ESR Normalization or decrease in white blood cell (WBC) count
Low platelets
Elevated liver enzymes
Increased ferritin
Increased triglycerides Low fibrinogen
Erratic fevers
Hemorrhages (disseminated intravascular
coagulation–like pattern) Prognosis
For oligoarthritis, it is good.
Condition may resolve on puberty
Polyarticular and systemic JIA ~ Still’s – bad
prognosis JIA in adolescence
Uveitis
Persistence into adulthood ~ 50% cases
Reduced peak bone mass
Adult onset Still’s disease
Rare systemic inflammatory disorder
Intermittent fever, rash and arthralgia
Hepato-splenomegaly and lymphadenopathy
may be present.
Acute phase response with marked elevation of
ferritin. RF and ANA negative FELTY’S SYNDROME A potentially serious condition
Associated with seropositive RA
Described in 1924
1-3% of all patients with RA
Female> Male Classical triad
Rheumatoid arthritis
Splenomegaly
Granulocytopenia
Death may happen due to infections
An eye to pathology
Abnormal splenic sequestration of
granulocytes
Destruction
Antibodies against granulocytes
Risk factors
RF positivity in high titers
Long-standing disease
Aggressive and erosive synovitis
Human leukocyte antigen (HLA)-DR4 positivity
and DR4 homozygosity
Extra-articular RA manifestations Major clinical features
Lymphocytic infiltration of lacrimal and salivary
glands- dry eyes and xerostomia
Named after Swedish opthalmologist Henrick
Sjogren
He introduced the term ‘Keratoconjunctivits sicca’,
for dryness of eyes. The pattern
On third of patients have systemic
manifestations
Small but significant number may develop
lymphoma. Primary – presenting alone
Secondary – with other autoimmune
rheumatism
Two age peaks, first in twenties and then in
sixties
Female to male 9:1
Pathologic hallmarks
T and B Lymphocytic infiltration of salivary
glands and B lymphocytic hyperactivity.
T cells predominate in mild lesions, B cells
in more severe lesions
Ductal and acinar epithelial cells play a
significant role. Signals for lymphocytic activation
Produce pro-inflammatory cytokines and
lympho-attractant chemokines
Express functional receptors for immunity
T and B Cells resist apoptosis
B Cell Activating Factor (BAFF) surge in
patients, which provides the anti apoptotic effect. Glandular epithelial cells produce BAFF, mostly activated by a coxsackie virus strains Why diminished secretions?
Defect in cholinergic activity
Redistribution of water channel protein
aquaporin -5
Both leads to neuro epithelial dysfunction
and hence diminished glandular secretions. Clinical manifestations
Functional Differences and Similarities in Activated Peripheral Blood Mononuclear Cells by Lipopolysaccharide or Phytohemagglutinin Stimulation Between Human and Cynomolgus Monkeys PDF
Functional Differences and Similarities in Activated Peripheral Blood Mononuclear Cells by Lipopolysaccharide or Phytohemagglutinin Stimulation Between Human and Cynomolgus Monkeys PDF
