Systemic sclerosis

Question 1

Limited cutaneous systemic sclerosis
1) What is normally the 1st sign
2) What antibodies is it associated with?
3) Scleroderma typically affects where?
3) A subtype of limited systemic sclerosis is CREST syndrome - what are the 5 features?

Answer 1

1) Raynaud’s phenomena
2) Anti-centromere antibodies
3) Face and distal limbs
4) Calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia

Question 2

Diffuse cutaneous systemic sclerosis
1) Where does the scleroderma typically affect?
2) What antibody is it associated with?
3) It has a poor prognosis - name 2 complications

Answer 2

1) Trunk and proximal limbs
2) Anti SCL-70
3) Hypertension, pulmonary hypertension, ILD, renal disease

Question 3

What antibody is positive in 90% of systemic sclerosis cases?

Answer 3

ANA (antinuclear)