Limited cutaneous systemic sclerosis — SCE Rheumatology MCQ

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EasyConnective tissue diseasesLimited cutaneous systemic sclerosisSCE Rheumatology

A 56-year-old woman has Raynaud phenomenon, gastro-oesophageal reflux and progressive tightening of her fingers. Examination shows sclerodactyly and telangiectasia. Skin thickening is confined to the fingers and distal forearms, with no proximal limb or truncal involvement. ANA is positive, with anti-centromere antibodies. What is the most likely diagnosis?

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Correct answer: A — Limited cutaneous systemic sclerosis

The diagnosis is limited cutaneous systemic sclerosis. Raynaud phenomenon, sclerodactyly, telangiectasia and reflux are characteristic systemic-sclerosis manifestations, while anti-centromere antibodies strongly support this phenotype. The key discriminator is that skin thickening remains distal to the elbows without proximal limb or truncal involvement. Diffuse cutaneous systemic sclerosis involves skin proximal to the elbows or knees and/or the trunk, although antibody associations are not absolute. Eosinophilic fasciitis typically causes painful symmetrical fascial induration with groove signs and usually spares the fingers. Mixed connective tissue disease is associated with high-titre anti-U1-RNP antibodies and overlapping connective-tissue-disease features. Primary Raynaud phenomenon cannot account for sclerodactyly, telangiectasia or systemic involvement.

Reference: Van den Hoogen F et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013;72:1747-1755. https://pubmed.ncbi.nlm.nih.gov/24092682/