The onset of MDA5-DM involves genetic predisposition, environmental triggers and autoimmune dysregulation, where the type I interferon (IFN-I) pathway, anti-MDA5 IgG and various immune cells may all participate.[4] Recent publications have suggested that over-activation of IFN-I signaling may drive vasculopathy, contributing to both cutaneous and pulmonary lesions.[2] Viral infection is thought to be a possible trigger for MDA5-DM. This evidence concerns the gene IFIH1 and dermatomyositis.