JUST SCLERODERMA AUTOAB

Question 1

Anti-RNA polymerase III

Answer 1

diffuse skin fibrosis
tendon friction rubs and joint contractures
SCLERODERMA RENAL CRISIS

Question 2

Scl-70 aka topoisomerase 1

Answer 2

diffuse cutaneous

Question 3

Centromere

Answer 3

reduced risk severe lung disease
neg association with scleroderma renal crisis
limited cutaneous in 60%

Question 4

Th/To

Answer 4

poor outcomes in limited cutaneous

pulmonary hypertension

Question 5

PM-SCL

Answer 5

Molymyostitis scleroderma overlap

Question 6

Anti-U3RNP

Answer 6

more often skeletal muscle involvement
more PAH
NOT associated with renal involvement

NOT the same as anti-U1 RNP which is seen in MCTD and SLE (in SLE usually in comb with other ab)

Question 7

scleroderma GIT involvement- how works?

Answer 7

Autonomic nerve involvement–>smooth muscle atrophy and eventual irreversible muscle fibrosis of the gut–>hypomotility

Presence of WIDE MOUTH DIVERTICULA is PATHOGNOMONIC OF SCLERODERMA
Compared with achalasia there is a decrease in the action of the LES