Work

Diagnostic and prognostic significance of myositis-specific autoantibodies in idiopathic inflammatory myopathies

Year: 2026

Type: article

Abstract: Idiopathic inflammatory myopathies (IIMs) are heterogeneous immune-mediated muscle disorders with variable extramuscular manifestations and outcomes. Myositis-specific auto-antibodies (MSAs) have transformed the IIM field by enabling a serology-informed taxonomy based on distinct clinical phenotypes, prognostic trajectories, and therapeutic responses. Anti-Jo-1 and other antisynthetase antibodies characterize interstitial lung disease (ILD)-prone forms of disease; anti-MDA5 identifies patients at risk of rapidly progressive ILD and early mortality; anti-TIF1-γ and anti-NXP2 are indicative of cancer-associated myositis; anti-SRP and anti-HMGCR delineate necrotizing myopathies; and anti-cN1A supports the diagnosis of inclusion-body myositis. Beyond diagnosis, MSA profiles provide prognostic information, including about survival, relapse risk, and organ-specific complications, and they are increasingly being used to guide treatment decisions and predict biologic responsiveness. This review summarizes the diagnostic, prognostic, and therapeutic implications of MSAs across the IIM spectrum and highlight their growing importance in clinical practice for risk stratification and patient management.

Source: Annals of Clinical Neurophysiology

DOI: 10.14253/acn.25017

Authors: Ji-Yon Kim, Hsueh-Wen Hsueh, Eun-Jae Lee, Hyunjin Kim, Young-Min Lim

Institutions: Asan Medical Center, University of Ulsan, National Taiwan University Hospital

Language: English

Topic: Inflammatory Myopathies and Dermatomyositis

Subfield: Epidemiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: diamond

OpenAlex ID: W7141198583