[CITATION][C] Inclusion body myositis and myopathies

RC Griggs, V Askanas, S DiMauro… - Annals of Neurology …, 1995 - Wiley Online Library
RC Griggs, V Askanas, S DiMauro, A Engel, G Karpati, JR Mendell, LP Rowland
Annals of Neurology: Official Journal of the American Neurological …, 1995Wiley Online Library
The term “inclusion body myositis”(IBM) was coined in 197 1 [11, although an earlier
description had already defined the characteristic pathological findings of the disease {2}.
The diagnosis of IBM was initially established on the basis of the distinctive
tubulofilamentcontaining inclusions among patients with “polymyositis”(PM). Later it became
clear that IBM was a distinctive clinicopathological entity 131. There is, in fact, little overlap of
the typical clinical and pathological findings among the three major idiopathic inflammatory …
The term “inclusion body myositis”(IBM) was coined in 197 1 [11, although an earlier description had already defined the characteristic pathological findings of the disease {2}. The diagnosis of IBM was initially established on the basis of the distinctive tubulofilamentcontaining inclusions among patients with “polymyositis”(PM). Later it became clear that IBM was a distinctive clinicopathological entity 131. There is, in fact, little overlap of the typical clinical and pathological findings among the three major idiopathic inflammatory myopathies: dermatomyositis, PM, and IBM C41. The identification of kindreds with “familial IBM” suggests that IBM might sometimes be a genetic disease. The rimmed vacuoles and tubulofilaments characteristic of IBM occur in several clinically different hereditary myopathies. However, inflammation is almost invariably seen in sporadic IBM but only rawly encotlntered in familial IBM. It is therefore appropriate to term the two disorders “sporadic inclusion body myositis”(s-IBM) and the “familial or hereditary inclusion body myopathies”(h-IBM). The authors focus on s-IBM and h-IBM and propose criteria for the definition of s-IBM developed at a recent meeting on the disorder that considered:(1) What are the clinical and laboratory criteria that define s-IBM and h-IBM?(2) Which are the most promising areas of investigative work?(3) What are the prospects for treatment? The clinical, electromyographic, and pathological features of IBM were recently reviewed 14-77.
Diagnostic Criteria (Jerry R. Mendell) The majority of s-IBM patients have the characteristic clinical and laboratory features outlined in Table 1 {4-73. Problems in diagnosis arise when patients have some, but not all of the findings. The most common scenario is a patient with all the typical features of the disease including an inflammatory myopathy who does not show vacuolated muscle fibers, intracellular amyloid deposits, or 15-to 18-nm tubulofilaments. Such patients could be misdiagnosed as having PM unless the diagnosis of s-IBM is carefully pursued by ob-
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