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Lupus nephritis — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologyLupus nephritisABIM Board

A 43-year-old woman has ulcerative Gottron papules, mechanic’s hands, minimal muscle weakness, positive anti-MDA5 antibody, and rapidly worsening hypoxemia with new diffuse ground-glass opacities over ten days. Infection is evaluated in parallel. Which management principle is most appropriate?

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Correct answer: ABegin 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/