Article

Evaluation and treatment of inflammatory myopathies

Department of Neurology, Brigham and Women's Hospital, Boston, MA 02115, USA.
Journal of neurology, neurosurgery, and psychiatry (Impact Factor: 5.58). 10/2009; 80(10):1060-8. DOI: 10.1136/jnnp.2008.169375
Source: PubMed

ABSTRACT The major types of idiopathic inflammatory myopathy include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune mediated necrotising myopathy (NM). These myositides appear clinically, histologically and pathogenically distinct. DM, PM and immune mediated NM are responsive to immunosuppressive therapy, in contrast with IBM which is generally refractory to therapy. Greater understanding of the pathogenic bases of these disorders should hopefully lead to better treatment. We need well designed, prospective, double blind, placebo controlled trials in order to determine the best therapeutic options for these different disorders.

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    • "The inflammation found in muscle biopsies of these patients was undistinguishable from that found in PM [4] [5]. Gradually, clinical differences between IBM and PM became more important [1,8–10] and some patients diagnosed with PM turned out to have IBM at follow up [11] [12] [13] [14] [15] raising doubt about the validity of endomysial localization of inflammation with invasion of non-necrotic fibers as a diagnostic criterion for PM [10] [16] [17]. We investigated the disease course in patients with endomysial mononuclear cell infiltrates with invasion of non-necrotic fibers, hypothesizing that their disease course is in keeping with IBM and not PM, even if they did not fulfill histopathological or clinical criteria for IBM at onset. "
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    ABSTRACT: The objective of this study was to investigate if patients with endomysial mononuclear cell infiltrates invading non-necrotic fibers have a disease course consistent with inclusion body myositis (IBM), irrespective of other histopathological and clinical characteristics. All patients with a muscle biopsy showing endomysial inflammation with invasion of non-necrotic muscle fibers during the period 1979-2006 in two tertiary neuromuscular referral centers were classified into three groups: 1) patients whose biopsies also showed rimmed vacuoles; 2) patients whose biopsies showed no vacuoles but fulfilled clinical criteria for IBM, and 3) patients whose biopsies showed no vacuoles, and also did not fulfill clinical criteria for IBM (unclassified patients). These groups were compared with regard to age, gender, clinical features, and disease course including response to immunosuppressive treatment. Eighty-one individuals (41 men) were included. Rimmed vacuoles were found in 49 patients (60.5%). Fourteen patients (17.3%) fulfilled clinical criteria for IBM and 18 patients (22.2%) were unclassified at presentation. At follow up (mean duration 9 years) three women remained unclassified (4%). There were no differences in disease course or effect of treatment between the three groups. Men had more often rimmed vacuoles than women (73% vs 48%; p = 0.018), and women more often than men were unclassified. Women tended to show more often temporary improvement if treated (p = 0.07), but none had sustained improvement. In conclusion, patients with a muscle biopsy showing endomysial cell infiltration with invasion of non-necrotic muscle fibers most probably have IBM, regardless of clinical and other pathological features. Women lack typical features more often than men. Copyright © 2015 Elsevier B.V. All rights reserved.
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