Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria

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Prevalence and incidence

A number of studies conducted in different countries have reported the incidence and prevalence of inflammatory myopathies and the methodology and results of these studies are summarized in Table 1. The majority of the more recent investigations have used the Bohan and Peter [1] diagnostic criteria for determining inclusion of cases. Two of the studies of inclusion body myositis (IBM) used criteria proposed by Griggs et al [2] and one used the European Neuromuscular Centre criteria [3]. All of

Autoimmune diseases

Inflammatory myopathies may occur in association with various types of autoimmune disorders. Myositis has been reported in 5% to 17% of patients with scleroderma, 4% to 16% of patients with systemic lupus erythematosus (SLE), and 16% to 79% of patients with mixed connective tissue disease [14], [15], [16], [17]. In some studies, the criteria for myositis were not sufficiently specific for diagnostic certainty.

A few studies have examined in more detail the association of myositis with

Classification

Although a number of IIM classification schemes have been proposed, none has been prospectively validated or carefully studied to determine its utility in practice. From a clinicopathologic perspective, the IIMs fall into six major categories: (1) dermatomyositis, (2) polymyositis, (3) overlap syndromes, (4) cancer-associated myositis, (5) inclusion body myositis, and (6) other forms including rare focal and diffuse variants (Box 1). Dermatomyositis is the most consistent entity, in terms of

Diagnostic criteria

Although the diagnosis of an IIM may be suspected on clinical grounds, and is supported by the finding of a raised serum creatine kinase level and abnormal electromyogram, a definitive diagnosis can only be made on the basis of histopathologic changes and requires a muscle biopsy. Although a raised serum CK level supports the diagnosis of an IIM, the test has a low specificity and may be normal, particularly in some patients with DM and IBM. Other serum enzyme levels, including aldolase,

Summary

Epidemiologic studies have helped to define the prevalence and incidence of PM, DM, and IBM and have highlighted differences in risk between men and women and in the age at onset for the different forms of myositis. Additionally, these studies have shown that there is a substantially higher risk of PM and DM in certain racial groups which is likely to be genetically determined. These differences are all likely to be fundamental in terms of the pathogenesis of these diseases but, as yet, their

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