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Dermatomyositis — SCE Dermatology MCQ

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HardConnective Tissue & VasculitisDermatomyositisSCE Dermatology

A 50-year-old woman with dermatomyositis has anti-MDA5 (anti-CADM-140) antibodies. What is the most important clinical association of this antibody?

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Correct answer: DRapidly progressive interstitial lung disease

Anti-MDA5 (melanoma differentiation-associated protein 5, also called anti-CADM-140) antibodies in dermatomyositis are strongly associated with clinically amyopathic dermatomyositis (minimal or absent muscle weakness) and rapidly progressive interstitial lung disease (RP-ILD), which carries high mortality. Aggressive immunosuppression with combination therapy (calcineurin inhibitors + cyclophosphamide + corticosteroids) is recommended at diagnosis. The characteristic skin features include skin ulceration, mechanic's hands, and palmar papules. This contrasts with anti-TIF1-gamma (cancer-associated) and anti-Mi-2 (good prognosis).

Reference: BAD 2016 DM Guidelines; Rheumatology 2019