Dermatomyositis — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesDermatomyositisSCE Rheumatology

A 52-year-old man presents with rapidly progressive symmetrical proximal muscle weakness and Gottron papules. His creatine kinase is 5300 IU/L, and adult-onset dermatomyositis is diagnosed. His myositis antibody profile is positive for anti-TIF1-gamma antibodies. Which associated complication should be prioritised for risk-stratified screening?

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Correct answer: A — Occult malignancy

Anti-TIF1-gamma-positive adult dermatomyositis is strongly associated with occult malignancy. This patient therefore requires prompt, risk-stratified cancer screening in addition to participation in routine population screening programmes. The cancer association is greatest around the onset of inflammatory myopathy. Rapidly progressive interstitial lung disease is more characteristic of anti-MDA5 dermatomyositis. Calcinosis is particularly associated with juvenile dermatomyositis and anti-NXP2 phenotypes. Myocarditis and oropharyngeal dysphagia are important potential manifestations of inflammatory myopathy and should be assessed when clinically indicated, but neither is the complication specifically signalled by anti-TIF1-gamma positivity.

Reference: Oldroyd AGS et al. International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an IMACS initiative. Nature Reviews Rheumatology. 2023;19:805-817. https://pubmed.ncbi.nlm.nih.gov/37945774/