Systemic Sclerosis — SCE Rheumatology MCQ

Correct answer: E — Selected early severe, rapidly progressive diffuse cutaneous disease with poor prognosis, without advanced cardiorespiratory involvement

Explanation lettering: C = shown as A · A = shown as C

E is correct. AHSCT is not routine for all diffuse cutaneous SSc and is not triggered by isolated skin disease or failure of a single csDMARD. It is reserved for highly selected patients with early severe, rapidly progressive diffuse cutaneous disease and a poor prognosis, before advanced cardiorespiratory damage makes transplantation unacceptably hazardous. Selection requires an expert SSc–transplant multidisciplinary team and detailed cardiopulmonary assessment. A is unsuitable because severe pulmonary hypertension and ventricular dysfunction substantially increase transplant risk. B lacks an aggressive diffuse phenotype. C ignores the required prognostic and safety selection. D applies an arbitrary one-drug threshold; response to conventional immunosuppression may inform decision-making but is not the defining indication. ASTIS and SCOT demonstrated long-term benefits over cyclophosphamide, offset by early treatment-related mortality and substantial toxicity.

Reference: Denton CP et al. The 2024 British Society for Rheumatology guideline for management of systemic sclerosis, section on autologous haematopoietic stem cell transplantation. Rheumatology. 2024;63:2956–2975. https://pubmed.ncbi.nlm.nih.gov/39255973/