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Systemic sclerosis — SCE Rheumatology MCQ

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EasyConnective Tissue DiseasesSystemic sclerosisSCE Rheumatology

A 46-year-old woman has Raynaud's phenomenon, reflux and progressive skin thickening of the fingers extending to the forearms. Nailfold capillaroscopy shows giant capillaries and dropout. ANA is positive with anti-topoisomerase I antibodies. FVC is 72% predicted. What is the most likely diagnosis?

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Correct answer: EDiffuse cutaneous systemic sclerosis

Explanation lettering: E = shown as A · A = shown as B · D = shown as C · B = shown as D · C = shown as E

Diffuse cutaneous systemic sclerosis is best because skin thickening proximal to the MCPs with anti-topoisomerase I and reduced FVC supports diffuse cutaneous systemic sclerosis. A is less suitable because limited disease usually remains distal to elbows and is often anticentromere-positive; B is less suitable because morphea is localised skin sclerosis without systemic vascular features; D is less suitable because eosinophilic fasciitis spares the fingers and is not associated with Raynaud's capillaroscopy changes; E is less suitable because MCTD is associated with high-titre anti-U1 RNP and overlap features. Clinical pearl: skin subset and antibody profile stratify organ-risk in systemic sclerosis.

Reference: BSR systemic sclerosis guideline 2024; EULAR systemic sclerosis recommendations 2024