What is another name for Sjögren’s syndrome?
Sicca syndrome
What is Sjögren’s syndrome?
Inflammatory destruction of the lacrimal and salivary glands
Primary effect of sjögren’s syndrome?
activated CD4 cells
Genetic factors of Sjögren’s syndrome?
HLA-DR3 and HLA-DR4 alleles
Forms of Sjögren’s syndrome?
- isolated disorder (Sicca)
- associated with other autoimmune disorders (SLE, RA)
Autoantibodies found in Sjögren’s syndrome (3)
- against ribonucleoprotein antigens : SSA (Ro) and SSB (La)
- RF in 75% patients
- ANAs in 50-80% of patients
Morphology of SS?
Lymphocyte and plasma cell infiltration that may form germinal centers
Clinical presentation of SS?
- dry eyes (keratoconjunctivitis sicca)
- dry mouth (xerostomia)
Types of sclerosis
- diffuse systemic sclerosis
- limited systemic sclerosis
Characteristic of diffuse systemic sclerosis? (3)
- disseminated skin involvement
- rapid clinical manifestation
- early GI involvement
Characteristics of diffuse systemic sclerosis (3)
- Restricted to face and fingers
- Late visceral involvement
- CREST
What does CREST stand for?
- calcinosis
- reynaud’s
- esophageal dysmobility
- sclerodactility
- telangiectasia
3 steps of pathogenesis of scleroderma
- autoimmune response
- vascular damage
- collagen deposit
ANAs in scleroderma? (2)
- anti DNA topoisomerase I
- anti centromere
Steps of autoimmunity in scleroderma (4)
- CD4 cell activation, cytokine release
- Th2 cells produce IL-13
- Macrophage activation : TGFb production
- Collagene synthesis, fibrosis
Other organs involved in scleroderma?
- GI - 90% of cases
- Lung - 50% of cases
- Kidney
- Heart
Clinical manifestation of scleroderma (3)
- Female / male ratio : 3:1
- Peak incidence over 50-60 years
- Reynaud in all patients
- Ondulent
- Dyspnoe : cor pulmonale
What type of autoimmunity is polyarteritis nodosa?
Type 3 hypersenstivity
What is polyarteritis nodosa?
Medium sized vessel vasculitis
Clinical features of PN?
- Skin symptoms
- Coronary artery disease
- Renal disease
- Polyneuropathy
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