Immunpathogenese und Therapie inflammatorischer Myopathien
| Autor: | R. Hohlfeld, H. Wiendl |
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| Rok vydání: | 2008 |
| Předmět: | |
| Zdroj: | Aktuelle Neurologie. 35:185-191 |
| ISSN: | 1438-9428 0302-4350 |
| DOI: | 10.1055/s-2007-986395 |
| Popis: | Inflammatory myopathies are currently subdivided into three main entities: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). This categorisation is based on a combination of clinical, histological and immunopathological characteristics. The classical pathogenic concepts assume that an immune-mediated damage by cytotoxic T cells plays a major role in polymyositis and in inclusion body myositis. In dermatomyositis, humoral components directed against capillaries or small vessels are considered to be instrumental. The pathogenetic importance of clonally expanded CD8 T cells in the pathogenesis of polymyositis as well as inclusion body myositis has been well validated by various studies over the last years. Furthermore, novel studies show the active role of the muscle in the direct interaction with immune cells and help to identify the potential therapeutic target structures for immune intervention. Recent years have brought forth novel findings, particularly concerning the pathogenesis of dermatomyositis, which broaden the hypothetical scenaries for pathogenesis that have been valid so far. While polymyositis and dermatomyositis are well controllable in most cases by means of conventional immunosuppressive strategies (above all, corticosteroids, azathioprine), the disease course of most cases of inclusion body myositis can hardly be modulated. Novel pathogenetic findings also have clear implications with regard to the possibilities of immunoselective interventions. This paper surveys the current status of the pathogenesis of and/or therapy for autoimmune inflammatory myopathies. |
| Databáze: | OpenAIRE |
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