Polymyositis (PM) and dermatomyositis (DM) are subtypes of autoimmune inflammatory myopathies. Interstitial lung disease (ILD) involvement is common in PM/DM. There is no evidence base for immunosuppression in DM/PM-ILD and current evidence is based on case stories and expert opinions. We present a 63-year-old male with severe respiratory failure due to PM-ILD who was treated successfully with intravenous immunoglobulin, recovered the acute phase and survived more than 4 years.

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