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Neuropathol Appl Neurobiol. 2017 Feb;43(1):82-91. doi: 10.1111/nan.12384.

Sporadic inclusion body myositis - a myodegenerative disease or an inflammatory myopathy.

Author information

1
Department of Neurology and Hope Center for Neurological Diseases, Washington University School of Medicine, Saint Louis, MO, USA.
2
Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.

Abstract

Sporadic inclusion body myositis (sIBM) is an insidious late-onset progressive myopathy that typically affects patients over the age of 50. Clinically, patients develop a characteristic pattern of weakness that affects the forearm flexors and knee extensors. Muscle biopsy, often utilized in the diagnosis, demonstrates a chronic myopathy with mixed pathologies harbouring intramyofiber protein inclusions and endomysial inflammation. The co-existence of these pathologic features (that is, inflammation and protein aggregation) has divided the field of sIBM research into two opposing (albeit slowly unifying) camps regarding disease pathogenesis. The present review explores the recent evidence supporting these distinct pathogenic mechanisms. Future therapies that are designed to target both aspects of sIBM pathologies will likely be necessary to treat sIBM.

KEYWORDS:

myositis

PMID:
28111778
DOI:
10.1111/nan.12384
[Indexed for MEDLINE]

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