Abstract Background Myositis‐specific autoantibodies (MSAs) and myositis‐associated autoantibodies (MAAs) have emerged as crucial biomarkers in idiopathic inflammatory myopathies (IIMs). Methods This review synthesizes recent research on MSAs and MAAs in various IIM subtypes. Results Specific autoantibodies correlate with distinct clinical manifestations and pathological features. For example, anti‐MDA5 antibodies are linked to rapidly progressive interstitial lung disease, while anti‐TIF1‐γ antibodies are associated with increased malignancy risk in adult dermatomyositis. Animal models have demonstrated the pathogenic potential of certain antibodies, such as anti‐TIF1‐γ, anti‐SRP, and anti‐HMGCR, in inducing experimental myositis. Conclusions Understanding the roles of MSAs and MAAs is crucial for elucidating disease mechanisms, developing targeted therapies, and improving patient outcomes. Further research is needed to fully characterize their functional implications and explore their potential as biomarkers for disease activity, prognosis, and treatment response.

Myositis‐specific/associated autoantibodies as diagnostic keys and disease drivers

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