SLE - features (8)
4/11 ACR criteria - SOAP BRAIN MD
- Serositis - pleruritic chest pain (plerual inflammation)
- oral ulcers
- Blood disorders - pancytopenia
- renal involvement
- ANA +ve
- Immune path - anti DsDNA, anti-smith, anti-histones
- Neuro symptoms
- Malar - butterfly rash
- Discoid rash
ANA ab in SLE - specificly what can the abs be? (3)
- Anti Ds DNA
- Anti-smith - most specific but only 30% pts have
- Anti-histone - drug related e.g. hydralazine
What happens to glomerulus in SLE?
Immune complex deposition results in thickened GBM - membranous GNpathy
What is this?
Libman Sacks endocarditis - strands of fibrin, neutrophils,lymphocytes (but no bacteria)
SLE: gender, ethnicity, HLA assc.
Autoimmune multisystem disorder, high incidence in those with complement def, can be drug induced. F> M, afroC.
HLA DR2/3 assc.
Scleroderma 'hard skin'.
Scleroderma also autoimmune disorder, get tight skin due to excess collagen deposition & fibrosis
- Calcinosis - painful lesions on tips of fingers
- Raynauds - triphasic colour change
- Esophageal dysmotility - also due to collagen deposition
- Sclerodactyl - tightening of skin of fingers (cant pull it)
Scleroderma: can be diffuse or limited - what are the differences (abs & distribution)
What is the pattern of immunofluorescence
- Diffuse - involves trunk, anti DNA topoisomerase (Scl70)
- limited form - anticentromere ab
nucleolar pattern of IF
What will histology of Scleroderma show? (2)
- Collagen excess deposition everywhere
onion skin thickening of arterioles - intimal thickening, present with renal crises - BP>200
Mixed connective tissue disorder has features of 4 disorders, what are they?
(myositis presents with prox muscle weakness & tenderness > raised CK)
Features of dermatomyositis (2)
- Heliotrope rash
- Gottron papules - erythematous rash over knuckles
Mixed connective tissue disorders have speckled ANA pattern