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Therapie der Myositiden

Therapy of myositis

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Zusammenfassung

Zu den idiopathischen inflammatorischen Myositiden zählen die Dermatomyositis (DM), Polymyositis (PM), die Einschlusskörperchenmyositis („inclusion body myositis“, IBM) und die nekrotisierende autoimmune Myopathie (NAM). Bei DM und PM werden initial Glukokortikosteroide empfohlen. Steroid-sparende Immunsuppressiva wie Azathioprin, Methotrexat oder Cyclosporin A werden bei ungenügendem Ansprechen verabreicht, bei drohenden Steroidnebenwirkungen oder wenn die initiale Prognose ungünstig ist. Die Therapie kann auch mit intravenösen Immunglobulinen (IVIG) eskaliert werden. Tacrolimus und Mycophenolat Mofetil (MMF) waren in kleineren Fallserien effektiv. Cyclophosphamid sollte therapierefraktären Patienten vorbehalten bleiben. Auch MMF kann mit IVIG kombiniert werden, um die Therapie zu intensivieren. Die Evidenz für Rituximab ist für den Routineeinsatz ungenügend. TNF-α-Inhibitoren und Plasmapherese haben nicht überzeugt. Die kutanen Manifestationen der DM reagieren auf Sonnenschutz, Antimalariamittel, topische Glukokortikosteroide sowie Calcineurininhibitoren. Bei der NAM sollten Statine abgesetzt und Prednison mit Immunsuppressiva initiiert werden. Bei der IBM wird ein Therapieversuch mit Prednison, Methotrexat oder Azathioprin bei Kreatinkinase-Erhöhung oder entzündlichem Infiltrat empfohlen. In jedem Stadium der Myositiden ist Physiotherapie nützlich.

Abstract

Idiopathic inflammatory myopathy consists of dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). At all stages of myositis, physiotherapy is effective in improving muscle strength, endurance and in maintaining joint motion. In DM and PM the therapy is initiated with glucocorticosteroids. Steroid-sparing agents (azathioprine, methotrexate and cyclosporin A) are added to prevent Cushing’s syndrome or an unsatisfactory response. Therapy can also be escalated with intravenous immunoglobulins. Tacrolimus and mycophenolate mofetil (MMF) were effective in small case series. Cyclophosphamide is restricted to patients not responding to previous agents. For treatment intensification immunoglobulins can also be combined with MMF. There is not enough evidence to routinely recommend rituximab. The results with TNF-alpha inhibitors and plasmapheresis were negative or inconsistent. In DM skin involvement responds to sun blockers, antimalarials, topical corticosteroids or calcineurin inhibitors. In NAM statins should be discontinued and treatment with prednisone and immunosuppressants initiated. In IBM a therapeutic trial with prednisone, methotrexate or azathioprine may be warranted, especially in cases in which the serum creatine kinase (CK) is elevated or an inflammatory infiltrate is present in the muscle biopsy.

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  1. Rheumatologische Universitätspoliklinik Basel, Felix Platter-Spital, Burgfelderstr. 101, 4012, Basel, Schweiz

    A.D. Keck &  U.A. Walker

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  2. U.A. Walker
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Correspondence to U.A. Walker.

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Keck, A., Walker, U. Therapie der Myositiden. Z. Rheumatol. 72, 227–235 (2013). https://doi.org/10.1007/s00393-012-1080-y

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