Systemic Sclerosis

Question 1

what is systemic scleorsis?

Answer 1

condition characterised by hardened, sclerotic skin and other connective tissues

Question 2

gender difference in systemic sclerosis?

Answer 2

4x more common in females

Question 3

three patterns of systemic sclerosis?

Answer 3

limited cutaneous sys scl
diffuse cutaneous sys scl
scleroderma

Question 4

what is limited cutaneous sys scl?

Answer 4

scleroderma which affects the face and distal limbs predominantly
subtype is CREST syndrome

Question 5

what is CREST syndrome

Answer 5

calcinosis
raynaud’s
oEsophageal dysmotility
sclerodactyly
telangiectasia

Question 6

what may be the first sign of limited cutaneous sys scl?

Answer 6

raynaud’s

Question 7

what antibodies a/w limited cutaneous sys scl?

Answer 7

anti-centromere

Question 8

what is diffuse cutaneous sys scl?

Answer 8

scleroderma affecting trunk and proximal lumbs predominantly

Question 9

what is the most common cause of death in diffuse cutaneous sys scl?

Answer 9

respiratory involvement - seen in 80% (ILD, PAH)

Question 10

complications of diffuse cutaneous sys scl?

Answer 10

respiratory - ild, pah
renal disease
hypertension

Question 11

how should diffuse cutaneous sys scl patients with renal complications be managed?

Answer 11

ace inhibitor

Question 12

what is the prognosis of diffuse cutaneous sys scl like?

Answer 12

poor

Question 13

what antibodies is diffuse cutaneous sys scl a/w?

Answer 13

scl-70

Question 14

what is scleroderma

Answer 14

tightening + fibrosis of skin
may manifest as plaques (morphoea) or linear

Question 15

what antibodies are a/w systemic sclerosis?

Answer 15

ana - 90%
anti-scl-70
anti-centromere
rf - 30%

Question 16

a severe sclerodermic renal crisis may also present with?

Answer 16

microangiopathic haemolytic anaemia

Question 17

what is eosinophilic fasciitis?

Answer 17

rare form of systemic sclerosis
red forearms + lower legs with pitting oedema + uneven skin
spares hands, NO RAYNAUD’S