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Entzündliche Muskelkrankheiten

Polymyositis, Dermatomyositis und Einschlusskörpermyositis

Inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis

  • Schwerpunkt: Muskelschmerz aus internistischer Sicht
  • Published:
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Zusammenfassung

Die Dermatomyositis, Polymyositis, Einschlusskörpermyositis und die Myositis-Overlap-Syndrome sind immunologische Systemkrankheiten unbekannter Ursache. Leitsymptome der Diagnostik sind die Muskelschwäche und die Erhöhung der Kreatinkinase im Serum. Die Muskelbiopsie ergibt den entscheidenden Befund für die klinisch-nosologische Diagnose. Extramuskuläre Manifestationen an der Haut, den Gelenken und an inneren Organen (Lunge, Herz) prägen die verschiedenen klinischen Erscheinungsformen der Dermato- und Polymyositis, sie fehlen bei der Einschlusskörpermyositis. Mit Ausnahme der Einschlusskörpermyositis finden sich myositisassoziierte Autoantikörper häufig und in Assoziation mit bestimmten klinischen Manifestationen (z. B. Antisynthetase-Syndrom). Die Neoplasierate ist mehrere Jahre nach Manifestation der Muskelkrankheit erhöht. Insbesondere bei der Polymyositis ist eine Differenzialdiagnose infektiöser, endokriner, metabolischer und neuromuskulärer Ursachen der Muskelkrankheit erforderlich. Glukokortikosteroide sind bei der Dermato- und Polymyositis die Therapeutika der ersten Wahl. Methotrexat, Azathioprin, Cyclophosphamid, i.v.-Immunglobuline und andere Substanzen werden bei besonderen Verlaufsformen und hohem Glukokortikoidbedarf eingesetzt.

Abstract

Dermatomyositis, polymyositis, inclusion body myositis and myositis overlap syndromes are systemic immune disorders of unknown origin with muscle weakness and elevated values of creatinkinase in the serum. Muscle biopsy is pivotal for a proper clinical diagnosis. Extramuscular findings at the skin, the joints or internal organs (lung, heart) are characteristic for the different clinical presentations of dermato- or polymyositis and are usually absent in inclusion body myositis. With the exception of inclusion body myositis myositis-associated autoantibodies are frequently present and associated with distinct clinical manifestations (e. g. antisynthetase syndrome). The rate of malignancy is elevated for several years after onset of myositis. Especially in polymyositis an appropriate differential diagnosis of infectious, endocrine, metabolic or neuromuscular causes of muscle disease is necessary. Glucocorticosteroids are the first choice of treatment in dermato- or polymyositis. Methotraxate, azathioprine, cyclophosphamamide, i.v. immunoglobulins and other drugs are used in diseases courses with continuous high dose requirement of corticosteroids.

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  1. Rheumaklinik und Rheumaforschungsinstitut Aachen

    E. Genth

  2. Rheumaklinik und Rheumaforschungsinstitut, Burtscheider Markt 24, 52066, Aachen

    E. Genth

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Genth, E. Entzündliche Muskelkrankheiten. Internist 46, 1218–1232 (2005). https://doi.org/10.1007/s00108-005-1496-4

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