Connective Tissue Disease, Amyloid, Sarcoid, Immune Related Multisystem Disorders Flashcards
Name an autoimmune condition with an organ specific with organ specific antibodies
Pernicious anaemia
Name an autoimmune condition with an organ specific without organ specific antibodies
Primary biliary cirrhosis
Name three multi-system autoimmune diseases (3)
Rheumatoid arthritis
Sjorgren’s syndrome
SLE
What systems are affected in SLE (8)
Skin Oral ulcers Joints Neurological Serositis Renal Haematological Immunological
What is positive in SLE
ANA (anti-nuclear antibody)
What auto-antibodies are present in SLE (3)
Anti-dsDNA
Anti-smith (against ribonucleoproteins)
Anti-histone (drug related e.g. hydralazine)
Where in the skin are auto-antibodies present
Dermal-epidermal junction
What is seen in histology of the glomerulus in SLE
Wire loops (thickened pink glomerular capillary loops due to immune complex deposition)
What is scleroderma (systemic sclerosis)
Fibrosis and excess collagen (localised form is called morphoea in the skin)
What is the immunoflouresence pattern for scleroderma
Nucleolar pattern
What antibodies are present in diffuse systemic sclerosis
DNA topisomerase (Scl70)
What antibodies are present in limited cutaneous systemic sclerosis
Anticentremere antibody
What is CREST syndrome present in
Limited cutaneous systemic sclerosis
What are the feature of CREST syndrome (5)
Calcinosis Raynauds Eosophageal dysmotility Sclerodactyly Telangiectasia
What are some clinical features of scleroderma on physical examination (5)
Scleroderma (tightened skin on hands and face)
Calcinosis - may be visible on ends of fingers (very painful)
Raynaud’s (blue - purple - white)
Nail fold capillary dilatation
Telangiecstasia
What is the pathology in the skin of scleroderma
Increased dermal collagen resulting in reduced skin elasticity
What causes oedophageal dysmotility in scleroderma
Collagen deposition in the submucosa causes fibrosis (leads to dysmotility)
What changes occur in the arteries (histologically) in scleroderma
Onion skin - intimal thickening of small arteries