Anti-MDA5 ILD — SCE Dermatology MCQ
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Correct answer: C — Anti-MDA5 is associated with rapidly progressive interstitial lung disease (RP-ILD) which can be fatal within months — urgent pulmonary assessment and aggressive treatment are required
Anti-MDA5 (melanoma differentiation-associated gene 5) antibodies in dermatomyositis are strongly associated with clinically amyopathic DM (CADM) AND rapidly progressive interstitial lung disease (RP-ILD). RP-ILD can progress from normal to fatal respiratory failure within weeks to months. Urgent HRCT chest and pulmonary function tests are mandatory. If ILD is detected, aggressive triple immunosuppressive therapy (high-dose corticosteroids + calcineurin inhibitor [Tacrolimus] + IV Cyclophosphamide) should be initiated immediately. Rituximab, IVIG, and plasma exchange are additional options. The mortality of anti-MDA5 RP-ILD without early aggressive treatment exceeds 50%. This is a dermatological emergency requiring multidisciplinary intensive management.
Reference: BAD 2016 DM; Rheumatology RP-ILD