The corresponding myositis subgroup and clinical features for each MSA have been relatively well-described and different outcomes have been recognized; e.g., (i) anti-Mi-2 is associated with a classic DM, and usually with a good prognosis [7]; (ii) anti-MDA5 is commonly observed in rapidly progressive interstitial lung disease (RP-ILD) and/or vasculopathy in a clinical context of amyopathic or hypo-myopathic DM [8]; and (iii) anti-TIF-1γ and NXP-2 are considered as a high-risk factors for malignancy [9]. Here, MORC3 is linked to dermatomyositis.