Mitochondrial abnormalities in idiopathic inflammatory myopathies.

Area of Science:

• Neurology
• Immunology
• Mitochondrial Biology

Background:

• Idiopathic inflammatory myopathies (IIMs) are autoimmune muscle disorders characterized by immune-mediated damage.
• Mitochondrial dysfunction is increasingly recognized as a key factor in IIM pathogenesis, contributing to muscle weakness and disease progression.

Purpose of the Study:

• To review current knowledge on mitochondrial dysfunction in IIMs.
• To explore mechanisms, histopathology, clinical implications, and therapeutic strategies targeting mitochondrial impairment in IIMs.

Main Methods:

• A narrative literature review was conducted using PubMed.
• Searches included terms like "inflammatory myopathies" and "mitochondrial abnormalities."
• Studies on mitochondrial structure, function, histopathology, autoantibodies, and therapies were analyzed.

Main Results:

• Mitochondrial dysfunction in IIMs involves impaired oxidative phosphorylation, oxidative stress, and DNA damage.
• Histopathological findings include specific fiber types and altered mitochondrial morphology, especially in inclusion body myositis.
• Autoantibodies targeting mitochondrial components are associated with severe or refractory disease.

Conclusions:

• Mitochondrial dysfunction is a core pathogenic mechanism in IIMs, linked to immune-mediated muscle damage.
• Combined targeting of inflammatory and mitochondrial pathways holds promise for personalized IIM therapies.