Systemic sclerosis

Question 1

What is it?

Answer 1

is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

Question 2

3 patterns of disease

Answer 2

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

Scleroderma (without internal organ involvement)

Question 3

what is the first sign of limited cutaneous systemic sclerosis

Answer 3

raynauds

Question 4

where does scleroderma affect in limited cutaneous systemic sclerosis

Answer 4

face and distal limbs predominately

Question 5

which antibodies is limited cutaneous systemic sclerosis associated with

Answer 5

anti-centromere antibodies

Question 6

what is the sub-type of limited systemic scelorisis

Answer 6

CREST syndrome

Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

Question 7

where is the sceleroderma affected in diffuse cutaneous systemic sclerosis

Answer 7

trunk and proximal limbs

Question 8

which antibodies is diffuse cutaneous systemic sclerosis associated with

Answer 8

scl-70 antiboides

Question 9

what is the most common cause of death in diffuse cutaneous systemic sclerosis

Answer 9

repertory involvement

Question 10

what is the prognosis like diffuse cutaneous systemic sclerosis

Answer 10

poor

Question 11

what are the features of scleroderma

Answer 11

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

Question 12

associated antibodies

Answer 12

ANA positive in 90%

RF positive in 30%

anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis

anti-centromere antibodies associated with limited cutaneous systemic sclerosis