Zusammenfassung
Wir stellen den Krankheitsfall eines 72-jährigen Patienten mit einer seit 10 Jahren bestehenden anti-PM-Scl positiven systemischen Sklerodermie in Assoziation mit einer Einschlusskörper-Myositis vor. Das gemeinsame Auftreten einer Dermatomyositis oder Polymyositis mit einer anti-PM-Scl positiven systemischen Sklerodermie wurde bereits ausführlich beschrieben, während die Assoziation mit einer Einschlusskörper-Myositis nach unserem Wissen bisher nur einmal berichtet wurde. Die Differenzierung einer Einschlusskörper-Myositis von anderen Formen entzündlicher Myopathien wie der Polymyositis oder Dermatomyositis ist von besonderer Bedeutung, da die Einschlusskörper-Myositis kaum auf immunsuppressive Therapien anspricht. Unser Fall unterstreicht, dass bei Patienten mit anti-PM-Scl positiver systemischer Sklerodermie und therapieresistenter Myopathie die Diagnose Einschlusskörper-Myositis berücksichtigt werden sollte.
Summary
We describe a 72- year-old patient with a ten year history of anti-PM-Scl positive systemic sclerosis associated with inclusion-body myositis. While the association of dermatomyositis and polymyositis with anti-PM-Scl positive systemic sclerosis is frequently reported, inclusion-body myositis was, to the best of our knowledge, only previously described once in association with anti-PM-Scl-positive systemic sclerosis. The distinction between inclusion-body myositis and other forms of inflammatory myopathy, like the histopathologically well distinguishable polymyositis or dermatomyositis, is relevant because of the poor response of inclusion- body myositis to immunosuppressive treatment. Our case underlines that in patients with anti-PM-Scl-positive systemic sclerosis and treatment resistant progressive myopathy the diagnosis of inclusion body myositis should be considered.
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Kim, S., Genth, E., Krieg, T. et al. PM-Scl-Antikörper positive systemische Sklerodermie assoziiert mit Einschlusskörper-Myositis. Z. Rheumatol. 64, 499–502 (2005). https://doi.org/10.1007/s00393-005-0664-1
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DOI: https://doi.org/10.1007/s00393-005-0664-1