A 70-year-old woman presents with proximal muscle weakness, elevated CK (8× ULN), heliotrope rash, Gottron's papules, and mechanic's hands. Anti-MDA5 antibody is negative. Anti-Mi-2 antibody is positive. What is the expected pulmonary pattern?

• A Rapidly progressive interstitial lung disease
• B Pulmonary arterial hypertension
• C Diffuse alveolar haemorrhage
• D Minimal or no interstitial lung disease ✓

Explanation

Anti-Mi-2 antibody in dermatomyositis is associated with the classic dermatomyositis phenotype (heliotrope, Gottron's papules), good response to immunosuppression, and notably a low risk of interstitial lung disease. Conversely, anti-MDA5 antibody is associated with clinically amyopathic dermatomyositis and rapidly progressive, often fatal ILD. Anti-synthetase antibodies (anti-Jo-1, anti-PL-7, etc.) are associated with the anti-synthetase syndrome including ILD, mechanic's hands, and inflammatory arthritis.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.