Sporadic inclusion body myositis – a myodegenerative disease or an inflammatory myopathy

C. C. Weihl, A. L. Mammen

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)82-91
Number of pages10
JournalNeuropathology and Applied Neurobiology
Volume43
Issue number1
DOIs
StatePublished - Feb 1 2017

Keywords

  • myositis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Neurology
  • Clinical Neurology
  • Physiology (medical)

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