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Systemic Sclerosis — SCE Rheumatology MCQ

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HardSystemic SclerosisSystemic SclerosisSCE Rheumatology

A specialist systemic sclerosis multidisciplinary team is considering tocilizumab as initial immunomodulatory treatment. According to the 2024 British Society for Rheumatology recommendations for SSc-associated interstitial lung disease, which phenotype most specifically supports considering tocilizumab as first-line treatment, independent of the extent of ILD on CT?

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Correct answer: E — Early diffuse cutaneous SSc with anti-topoisomerase-I antibodies and raised inflammatory markers

The BSR guideline identifies early diffuse cutaneous SSc with raised inflammatory markers and anti-topoisomerase-I antibody positivity as the phenotype in which tocilizumab may be considered first-line, independent of the extent of ILD on CT. Mycophenolate remains the usual first-line treatment for SSc-ILD overall. Tocilizumab or rituximab may instead be added as rescue therapy when disease progresses on background immunosuppression, making D incorrect as a description of this specific first-line phenotype. PAH, isolated Raynaud phenomenon and anti-centromere-positive limited disease are not indications for first-line tocilizumab. The focuSSced trial did not meet its primary skin-score endpoint; the principal supportive signal was preservation of FVC in an early inflammatory SSc population.

Reference: British Society for Rheumatology. The 2024 British Society for Rheumatology guideline for management of systemic sclerosis, section: treatment of SSc-associated interstitial lung disease. 2024. https://pubmed.ncbi.nlm.nih.gov/32866440/