Lec 17 Scleroderma Flashcards
Who gets systemic sclerosis?
30-50 yo
women > men
blacks more than whites
What are early clinical features of scleroderma?
- raynauds
- edematous pahse
- arthralgias
- skin thickening on distal fingers
What serologic abnormalities do you see in scleroderma?
- ANA positive
- anti-Scl-70
What is string of perals sign?
asymptomatic but pathognemonic for scleroderma
When do you get scleroderma renal crisis?
stimulated by cold weather –> get intra-renal raynauds
treat w/ ACE inhibitors
What is diffuse scleroderma?
widespread skin involvement; rapid progression; early visceral invovlement
SCL-70 antibody
What is CREST variant?
- Calcinosis
- Raynaud
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia = red dots everywhere
anti-Centromere antibody
more benign than diffuse
What is treatment of scleroderma?
D penicillamine
What is mech of D penicillamine?
prevents collagen cross linking –> marked skin regression and increased survival
What are 5 inflammatory complicatsions of scleroderma?
- synovitis
- interstitial pneumonitis
- pericarditis
- myocarditis
- myositis
What is linear scleroderma?
straight line involved
What is morphea scleroderma?
have round patches
What is linear scleredema?
in pts with diabetes
What is eosinophilic fascitis?
occurs after exertion –> extremity pain and swelling followed by inudration
have increased ESR
assocaited w/ malignancy
What is nephrogenic systemic fibrosis?
rapidly progressive distal skin/visceral fibrosis
in pts on dialysis
