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  • Updated 05.03.2021
  • Released 03.23.1994
  • Expires For CME 05.03.2024

Inclusion-body myositis



Inclusion-body myositis is the most frequent and disabling myopathy seen in patients over 45 to 50 years of age. The distinct clinical features that lead to correct diagnosis and the inclusion-body myositis mimics are highlighted. Inclusion-body myositis has a complex pathogenesis in which autoimmune and inflammatory features coexist with elements of degeneration and abundant accumulations of various stressor proteins. In this article, the author discusses the pitfalls in diagnosis, the diagnostic markers, and the role of inflammation and degeneration in the pathogenesis of the disease, including the interaction between these processes. The latest trends in therapeutic strategies are also presented.

Historical note and terminology

Inclusion-body myositis (IBM) was first recognized as a distinct entity between 1967 and 1971. Three groups identified distinct microtubular filamentous inclusions by electron microscopy in the muscle biopsies of some patients with presumed polymyositis (28; 23; 150). The term "inclusion-body myositis" was coined to stress the uniqueness of the disease and separate it from polymyositis (150). Carpenter and colleagues’ seminal paper emphasizing the clinical entity (22) brought the disease to the surface and stimulated the interest of a number of investigators, including the author of this article.

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