Lupus nephritis — ABIM Board MCQ
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Correct answer: A — Begin urgent specialist combination immunosuppression for rapidly progressive ILD
The best answer is “Begin urgent specialist combination immunosuppression for rapidly progressive ILD”. Anti-MDA5 dermatomyositis can be clinically amyopathic yet cause fulminant, high-mortality interstitial lung disease. Rapid progression requires immediate multidisciplinary rheumatology, pulmonology, and critical-care treatment, commonly high-dose glucocorticoids combined early with agents such as cyclophosphamide, calcineurin inhibition, or rituximab according to the specialist protocol. Infection must be assessed concurrently, but little muscle weakness and a normal creatine kinase do not imply benign pulmonary disease.
Reference: ACR/CHEST Guideline for Treatment of Systemic Autoimmune Rheumatic Disease–ILD: https://pubmed.ncbi.nlm.nih.gov/38087426/