Scleroderma

Question 1

Localized scleroderma: differences from SSc

Answer 1

skin only, localized, no autoimmunity

Question 2

what is SSc

Answer 2

autoimmune, circulatory (small vessels), fibrotic, high mortality (no drugs)

Question 3

Four cell types involved in SSc

Answer 3

fibroblasts, endothelial cells, B and T cells

Question 4

limited SSc

Answer 4

early raynaud, anti-centromere ab’s, no renal crisis. Sclerosis of mostly distal skin

Question 5

diffuse SSc

Answer 5

skin before raynaud, early pulm(fibrosis&htn) cardiac and renal involvement. Anti-topo1 and RNA pol3

Question 6

Labs in dcSSc, biopsy

Answer 6

ANA, lowered PFTs. perivascular inflammation and dermal sclerosis

Question 7

vascular disease path

Answer 7

bland (non-inflamm) intimal proliferation

Question 8

topo1

Answer 8

diffuse; pulmonary fibrosis and cardiac involvement

Question 9

RNA poll 3

Answer 9

diffuse; extensive skin, SRC

Question 10

centromere (CENP/B,C)

Answer 10

limited, digital ischemia

Question 11

leading cause of dealth in SSc

Answer 11

ILD, pulmonary fibrosis. Do PFTs and HRCT

Question 12

Scleroderma renal crisis: when? result? cause?

Answer 12

occurs early, accelerated HTN. Onion skinning proliferation of intima, RBCs lyse as they squeeze thru

Question 13

treatment for pulm artery htn

Answer 13

endothelin1 blockers