Evaluation and treatment of inflammatory myopathies
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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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.Neuromuscular Disorders 03/2015; 25(6). DOI:10.1016/j.nmd.2015.02.014 · 3.13 Impact Factor
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ABSTRACT: Polymyositis and dermatomyositis are inflammatory myopathies that differ in their clinical features, histopathology, response to treatment, and prognosis. While their clinical pictures differ, they both present with symmetrical, proximal muscle weakness. Treatment relies mainly upon empirical use of corticosteroids and immunosuppressive agents. A deeper understanding of the molecular pathways that drive pathogenesis, careful phenotyping, and accurate disease classification will aid clinical research and the development of more efficacious treatments. This review discusses current knowledge of the epidemiology, clinical characteristics, diagnostic evaluation, classification, pathogenesis, treatment, and prognosis of polymyositis and dermatomyositis. This article is protected by copyright. All rights reserved.Muscle & Nerve 01/2015; 51(5). DOI:10.1002/mus.24566 · 2.31 Impact Factor
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ABSTRACT: We report a 49-year-old patient with necrotizing myopathy and a right renal mass. After laparoscopic radical nephrectomy, a remission of myopathy was seen. Pathologic evaluation of the nephrectomy specimen revealed a clear cell renal cell carcinoma. Relapse of myopathy 6 months postoperatively coincided with the diagnosis of the appearance of liver metastatic disease. After initiation of treatment with an mTOR-inhibitor, myopathy became less active requiring smaller amounts of corticosteroids with a complete remission of myopathy after 3 months of systemic treatment for metastatic renal cell cancer.09/2014; DOI:10.1179/2295333714Y.0000000080