PubMed · 5942304
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P Le Coulant, L Texier, J Maleville, J M Tamisier, M Géniaux, J P Denef. [Poikilodermatomyositis].. https://pubmed.ncbi.nlm.nih.gov/5942304/
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The treatment of the immune-mediated inflammatory myopathies remains largely empirical. Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add-on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low-dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3-6-month trial of such therapy is justified in this condition. More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms.
The techniques of magnetic resonance imaging and spectroscopy have been shown to have utility in the diagnosis and management of inflammatory muscle diseases. But perhaps more important have been the new insights into the pathophysiology of these diseases which MR studies, along with new immunologic data on autoantibodies and cellular infiltrates, have afforded. Pathologic subsets of inflammatory muscle disorders have been identified, suggesting, for example, that PM and DM are distinct disorders, thereby challenging the idea that these are relatively homogeneous syndromes. Further insights into disease pathogenesis which are likely to emerge from these new findings may allow identification of etiologic factors and improved approaches to treatment.
The inflammatory myopathies are a heterogeneous group of muscle diseases characterized by muscle degeneration mediated by inflammatory processes. They may be idiopathic, as in polymyositis, dermatomyositis and inclusion body myositis, or associated with systemic disorders such as malignancies, overlap syndromes, and retroviral infection. The pathogenesis of each disease is discussed together with more recent molecular and cellular immunology findings. Salient diagnostic, clinical and pharmacological features are also reviewed.