Sporadische Einschlusskörpermyositis

Zusammenfassung

Die sporadische Einschlusskörpermyositis (s-IBM) ist eine chronisch progressive entzündliche Myopathie, die im Wesentlichen jenseits des 50. Lebensjahres auftritt und histologisch durch »rimmed vacuoles« und eosinophile zytoplasmatische Einschlüsse gekennzeichnet ist. Die Ätiologie ist bislang unklar, verschiedene Mechanismen wie Slow-Virus-Infektion, Autoimmunpathogenese, Kernmatrixstörung und Störungen auf mitochondrialer Ebene werden diskutiert. Des Weiteren wird die s-IBM zunehmend in die Nähe von neurodegenerativen Erkrankungen und Prionerkrankungen gerückt. Therapeutische Möglichkeiten sind rar. Immunsuppressiva haben sich als unwirksam erwiesen, eine Stabilisierung des Krankheitsverlaufs ließ sich bislang nur durch hoch dosierte Immunglobulintherapie (IVIG) erreichen. Ein besseres Verständnis der Pathogenese könnte in Zukunft zu verbesserten therapeutischen Optionen führen.

Summary

Sporadic inclusion body myositis (s-IBM) is a chronic progressive inflammatory myopathy which preferentially occurs in older patients. Histologic hallmarks are rimmed vacuoles and eosinophilic cytoplasmatic inclusions. The etiology is still unknown, different pathogenetic mechanisms such as slow-virus-infection, autoimmunpathogenesis, myonuclear alterations and mitochondrial defects were implicated. A relation to neurodegenerative disorders and prion diseases was also suggested. There is a poor if any response to immunosuppressive therapy, stabilization of disease progression was shown by IVIG therapy, only. Future findings in the field of s-IBM pathogenesis may result in better therapeutic options.

Schlüsselwörter

Einschlusskörpermyositis - s-IBM - entzündliche Myopathie - Therapie

Keywords

Inclusion body myositis - s-IBM - inflammatory myopathy - therapy

• Literatur

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