Mitochondrial pathology in immune and inflammatory myopathies
- 1 November 2010
- journal article
- review article
- Published by Ovid Technologies (Wolters Kluwer Health) in Current Opinion in Rheumatology
- Vol. 22 (6), 651-657
- https://doi.org/10.1097/bor.0b013e32833f108a
Abstract
Acquired immune and inflammatory myopathies (IIMs) are typically subdivided into dermatomyositis, polymyositis and inclusion body myositis. However, many types of IIMs do not fit well into this scheme. Several myopathologic and autoantibody features of IIMs, that are not considered in standard classifications, are useful for defining individual disorders. We will review one set of myopathologic features that occur in some IIMs, mitochondrial abnormalities, and consider its diagnostic, treatment-related and pathogenic implications. Myopathologic changes that indicate mitochondrial disorders are often widespread in regions of muscle fiber abnormality in dermatomyositis. They distinguish dermatomyositis with vascular pathology from other inflammatory myopathies with skin changes that have prominent perimysial connective tissue lesions, but no mitochondrial, abnormalities. Mitochondrial abnormalities in scattered muscle fibers occur in sporadic inclusion body myositis and clinically similar disorders. Mitochondrial abnormalities in scattered nonnecrotic muscle fibers in IIM biopsies predict a poor response to immunosuppression. Muscle biopsy, including evaluation of mitochondrial stains, is important for the correct diagnosis of inflammatory myopathies. By recognizing the full range of distinctive myopathologic changes in the diverse group of IIMs, the clinician can improve diagnostic accuracy and apply appropriate treatment.Keywords
This publication has 56 references indexed in Scilit:
- Mitochondrial fission and remodelling contributes to muscle atrophyThe EMBO Journal, 2010
- Mitochondrial Fusion Is Required for mtDNA Stability in Skeletal Muscle and Tolerance of mtDNA MutationsCell, 2010
- Interferon‐stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophyAnnals of Neurology, 2010
- Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatmentBest Practice & Research Clinical Rheumatology, 2009
- Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathyBrain, 2009
- Autoantibodies to a 140‐kd protein in juvenile dermatomyositis are associated with calcinosisArthritis & Rheumatism, 2009
- Mouse models of mitochondrial DNA defects and their relevance for human diseaseEMBO Reports, 2009
- Identification of quantitative trait loci controlling cortical motor evoked potentials in experimental autoimmune encephalomyelitis: correlation with incidence, onset and severity of diseaseHuman Molecular Genetics, 2005
- Polymyositis with cytochrome oxidase negative muscle fibres. Early quadriceps weakness and poor response to immunosuppressive therapyBrain, 1997
- Microvascular Deposition of Complement Membrane Attack Complex in DermatomyositisNew England Journal of Medicine, 1986