Journal article
Inclusion body myositis: The interplay between ageing, muscle degeneration and autoimmunity
Best Practice & Research Clinical Rheumatology, Vol.36(2), Art. 101761
2022
Abstract
Inclusion body myositis (IBM) is a slowly progressive muscle disease affecting ageing individuals. IBM presents with a distinctive pattern of weakness involving the quadriceps and finger flexor muscles, although other muscles including pharyngeal muscles become affected over time. Pathological hallmarks of IBM include autoimmune features, including endomysial infiltration by highly differentiated T cells, as well as degenerative features marked by intramyofibre protein aggregates organised into inclusion bodies. Despite some progress in understanding the cellular pathways involved in IBM, it remains untreatable, and the progression of the disease leads to progressive weakness, disability, wheelchair dependency and loss of independence. Therefore, there is an urgent need to improve our understanding of the underlying mechanisms and pathways involved in this disease to identify new treatment targets. Here, we discuss the current understanding of aetiopathogenesis, the interrelationship between autoimmunity and degeneration, and how ageing is a major influencer of both these features.
Details
- Title
- Inclusion body myositis: The interplay between ageing, muscle degeneration and autoimmunity
- Authors/Creators
- E. McLeish (Author/Creator) - Murdoch UniversityN. Slater (Author/Creator) - Murdoch UniversityA. Sooda (Author/Creator) - Murdoch UniversityA. Wilson (Author/Creator) - Johns Hopkins MedicineJ.D. Coudert (Author/Creator) - The University of Notre Dame AustraliaT.E. Lloyd (Author/Creator) - Johns Hopkins MedicineM. Needham (Author/Creator) - Centre for Molecular Medicine and Innovative Therapeutics, Murdoch University, Perth, WA, Australia; Perron Institute for Neurological and Translational Science, Perth, WA, Australia; School of Medicine, University of Notre Dame, Fremantle, WA, Australia; Fiona Stanley Hospital, Department of Neurology, Perth, WA, Australia.
- Publication Details
- Best Practice & Research Clinical Rheumatology, Vol.36(2), Art. 101761
- Publisher
- Elsevier Ltd.
- Identifiers
- 991005541989407891
- Copyright
- © 2022 The Authors.
- Murdoch Affiliation
- Centre for Molecular Medicine and Innovative Therapeutics
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- International collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.106 Rheumatology
- 1.106.1684 Dermatomyositis
- Web Of Science research areas
- Rheumatology
- ESI research areas
- Clinical Medicine