Diagnostic and prognostic significance of myositis-specific autoantibodies in idiopathic inflammatory myopathies
- Author:
Ji-Yon KIM
1
;
Hsueh-Wen HSUEH
;
Eun-Jae LEE
;
Hyunjin KIM
;
Young-Min LIM
Author Information
- Publication Type:Review Article
- From: Annals of Clinical Neurophysiology 2026;28(1):33-49
- CountryRepublic of Korea
- Language:English
- 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.
