MYOSITIS AND MYOPATHIES: Edited by Andrea Doria, Anna Ghirardello and Mariele GattoAnti-MDA5 dermatomyositis: an update from bench to bedsideFuzzi, Enrico; Gatto, Mariele; Zen, Margherita; Franco, Chiara; Zanatta, Elisabetta; Ghirardello, Anna; Doria, Andrea Author Information Division of Rheumatology, Department of Medicine DIMED, Padua University Hospital, Padua, Italy Correspondence to Andrea Doria, Head of Rheumatology Unit, Department of Medicine, University of Padova, Padua 35128, Italy. E-mail: [email protected] Current Opinion in Rheumatology: November 2022 - Volume 34 - Issue 6 - p 365-373 doi: 10.1097/BOR.0000000000000908 Buy Metrics Abstract Purpose of review This review summarizes the recent developments about anti-MDA5 antibody positive dermatomyositis with a focus on its pathogenesis, clinical features and treatment options of rapidly progressive interstitial lung disease, its most ominous complication. Recent findings Anti-MDA5+ dermatomyositis has a heterogeneous clinical spectrum with different patient subsets exhibiting widely different outcomes; severe acute interstitial lung disease is the main factor impacting prognosis. The pathogenetic role of anti-MDA5 antibodies is an active area of investigation. Summary Anti-MDA5+ dermatomyositis has a wider spectrum of manifestations than previously thought. A high index of suspicion is needed not to miss atypical presentations. In the setting of acute interstitial lung involvement, once a confident diagnosis is made, an aggressive approach with early combined immunosuppression affords the best chances of survival. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.