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Inclusion body myositis (IBM) is the most common inflammatory myopathy after age 50 years.
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Despite similarities with polymyositis (PM) inflammatory disorders, IBM histopathology shows marked degeneration and protein aggregation.
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The clinical phenotype of typical IBM is distinctive, manifesting as proximal leg or distal arm weakness, although in our experience there are several phenotypic variants.
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IBM is refractory to all known immunosuppressive therapies.
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Low-intensity exercise may slow the rate
Inclusion Body Myositis
Section snippets
Key points
Epidemiology
Inclusion body myositis (IBM) is a rare sporadic disorder with a male/female ratio of 2:1 to 3:1. Data on prevalence and incidence of IBM vary depending on methodology, countries, and regions. In Western Australia, the overall prevalence was 9.3 per million, whereas the age-adjusted prevalence of IBM in people more than the age of 50 years is 3.5 per 100,000, making it the most common idiopathic inflammatory myopathy (IIM) in this age group.1 A recent study from South Australia yielded a higher
Diagnostic and research criteria
A multitude of IBM criteria have been proposed based on a variety of clinical and histopathologic features. Although these have been advanced for research use, they have permeated into the clinical realm. This article limits discussion to the 4 most prominent criteria sets, namely those by Griggs, the ENMC 2000, MRC 2010, and ENMC 2011. The 1995 Griggs IBM criteria represent the first major effort to define diagnostic criteria for IBM and are heavily weighted toward muscle pathology because
Summary
IBM is the most common inflammatory myopathy after age 50 years. Despite similarities with PM inflammatory disorders, IBM histopathology shows marked degeneration and protein aggregation. The clinical phenotype of typical IBM is distinctive, manifesting as proximal leg or distal arm weakness, although in our experience there are several phenotypic variants. IBM is refractory to all known immunosuppressive therapies. Low-intensity exercise may slow the rate of functional decline. Patients with
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This work was supported by an Institutional Clinical and Translational Science Award, NIH/National Center for Advancing Translational Sciences grant number UL1TR000001. The contents of this article are solely the responsibility of the authors and do not necessarily represent the official views of the NIH.