A distinct subtype of dermatomyositis (DM) characterized by high incidence of interstitial lung disease (ILD), skin ulceration and the absence of muscle weakness has been associated with circulating antibodies against melanoma differentiation-associated protein 5 (MDA5).[1] In this subtype of DM with autoantibodies to MDA5, ILD is present in 50%–100% cases and significantly contributes to elevated disease mortality.[1]. This evidence concerns the gene IFIH1 and interstitial lung disease.