Autoimmune myopathies

Dermatomyositis (DM) can be associated with the anti-melanoma differentiation-associated gene 5 (MDA5, IFIH1) autoantibody. Of note, patients present with severe extramuscular (notably lung and skin) manifestations and myopathy can be mild or missing, hence termed ``amyopathic'' DM previously. Interstitial lung disease is the leading cause of death in MDA5-DM, and death occurs frequently within one year after diagnosis in roughly 30% of these DM patients. We hereby report the autoptic findings of two male patients (30 and 42 years old) who succumbed to severe ILD in MDA5-related DM.

Source: Neuromuscular Disorders - September 28, 2020 Category: Neurology Authors: B. Englert, W. Stenzel Source Type: research