Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies. Interstitial lung disease (ILD) develops in most patients with DM and PM directly related to morbidity and mortality. Diagnosis requires a myositis panel and high-resolution computed tomography (HRCT). Prognosis depends on specific myositis-specific antibodies and the pattern of the interstitial lung changes. Anti-Mi-2 antibody-specific dermatomyositis has a lower prevalence of interstitial lung disease and has a favorable prognosis, responding well to steroids. Our patient is a 72-year old male who presented with recurrent episodes of pneumonitis, and ILD was found to have anti-Mi-2 beta-specific dermatomyositis and SLE overlap disease. He was responding well to high-dose steroids but was rebounding to similar symptoms whenever steroid weaning was attempted. He was started on azathioprine, but unfortunately, his disease rapidly progressed, and he died within a few months. This manuscript enhances the temporal relationship between dermatomyositis and ILD.
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