Immunotherapies in autoimmune inflammatory myopathies: Rationale and therapeutic updates

Area of Science:

• Neurology
• Immunology
• Rheumatology

Background:

• Autoimmune inflammatory myopathies (AIM) are a heterogeneous group of acquired muscle disorders characterized by endomysial inflammation.
• Accurate diagnosis is crucial due to clinical and immunopathologic heterogeneity among subtypes like dermatomyositis, necrotizing autoimmune myositis, antisynthetase syndrome-overlap myositis, and inclusion body myositis.

Purpose of the Study:

• To review the clinical and histopathologic features of AIM subtypes.
• To elucidate the immunopathogenic mechanisms underlying each subtype.
• To focus on applied immunotherapies and evidence-based efficacy from clinical trials.

Main Methods:

• Review of clinical and histopathologic features of AIM subtypes.
• Analysis of immunopathogenic mechanisms.
• Summary of applied immunotherapies and controlled clinical trials.
• Discussion of ongoing and future clinical trials.

Main Results:

• Advances in immunopathologic characterization have identified mechanism-specific AIM subsets.
• Clinical trials with biologic agents and monoclonal antibodies have enhanced understanding of pathogenic markers (T-cells, B-cells, cytokines, complement, antibodies, innate immunity).

Conclusions:

• Targeted immunotherapies based on specific AIM subsets hold promise for better clinical outcomes.
• Ongoing trials are exploring novel interventions for refractory AIM, emphasizing early application for promising future results.