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Anti-Synthetase ILD Treatment — SCE Rheumatology MCQ

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ModerateMyositisAnti-Synthetase ILD TreatmentSCE Rheumatology

A 45-year-old woman with dermatomyositis develops progressive dyspnoea and dry cough. HRCT shows bilateral lower lobe ground-glass changes with subpleural consolidation consistent with organising pneumonia pattern. Anti-Jo-1 is positive. What treatment approach is most appropriate for this ILD pattern?

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Correct answer: CGlucocorticoids often combined with Mycophenolate or Azathioprine for steroid-sparing

ILD in anti-synthetase syndrome frequently shows an NSIP or organising pneumonia pattern on HRCT. Glucocorticoids are the first-line treatment often producing good initial response particularly for organising pneumonia pattern. However relapse on steroid tapering is common so steroid-sparing agents (Mycophenolate Azathioprine or Rituximab) should be started early. For progressive fibrotic disease Nintedanib can be added as an anti-fibrotic agent. Cyclophosphamide may be considered for rapidly progressive ILD. The organising pneumonia pattern generally responds better to immunosuppression than UIP.

Reference: BSR 2022 myositis guidelines; Connors GR et al 2010 anti-synthetase syndrome ILD