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Aktuelle Rheumatologie 2016; 41(01): 59-66
DOI: 10.1055/s-0041-107939
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Differenzialdiagnose mittels Histopathologie bei Myositiden

Using Histopathology for the Differential Diagnosis of Inflammatory Myopathies
D. Pehl
1   Institut für Neuropathologie, Charité – Universitätsmedizin Berlin, Berlin
,
H.-H. Goebel
1   Institut für Neuropathologie, Charité – Universitätsmedizin Berlin, Berlin
,
W. Stenzel
1   Institut für Neuropathologie, Charité – Universitätsmedizin Berlin, Berlin
› Author Affiliations
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Publication History

Publication Date:
16 February 2016 (online)

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Zusammenfassung

Inflammatorische Myopathien sind zwar insgesamt relativ selten, stellen dabei jedoch aktuell die größte Gruppe potentiell behandelbarer Krankheiten der Muskulatur dar. Sie spielen im rheumatologischen Krankengut ebenso wie im neurologischen Krankengut sowohl im Kindes- wie auch im Erwachsenenalter eine signifikante Rolle, wobei das Management dieser Patienten häufig eine interdisziplinäre Herangehensweise erfordert. Es handelt sich um eine ganz heterogene Gruppe von Erkrankungen, die traditionell in Dermatomyositis, Polymyositis, und später, auch sporadische Einschlußkörperchen Myositis gruppiert wurden. In den vergangenen Jahren sind nun eine Vielzahl von neuen Erkenntnissen zur Pathogenese, zu Autoantikörpern, zur Beteiligung verschiedener Organe usw. erhoben worden, so dass sich dieses simplifizierte Konzept nicht mehr aufrechterhalten lässt. Auf der Basis dieser aktuellen Erkenntnisse sind neue Klassifikationssysteme entwickelt und weiterentwickelt worden. Heute werden 6 Entitäten voneinander unterschieden: Dermatomyositis (DM), Polymyositis (PM), Autoimmun vermittelte Nekrotisierende Myositis (ANM), Anti-Synthetase-Syndrom-assoziierte Myositis (ASS-assoziierte Myositis) und unspezifische Myositis (NSM), sowie die sporadische Einschlusskörperchen Myositis (sIBM) ([Stenzel 2015], [Hoogendijk 2004]). Diese Gruppen sind bis auf die sIBM auch im Kindesalter, dort jedoch mit einem anders gewichteten Verteilungsmuster der einzelnen Subentitäten als im Erwachsenenalter anzutreffen ([Rider 2013]). Die Unterscheidung zwischen diesen Kategorien hat fundamentale Bedeutung für die Prognose und das Therapieansprechen, so dass es äußerst wichtig erscheint, sie korrekt zu identifizieren, zu benennen und von anderen Erkrankungen, die klinisch ähnlich erscheinen, abzugrenzen. In diesem Übersichtsartikel möchten wir das aktuelle Wissen über die erwähnten Entitäten unter klinisch-pathologischen Gesichtspunkten zusammenfassen und die Histopathologie dieser Erkrankungen illustrieren.

Abstract

Although inflammatory myopathies are relatively rare overall, they are currently the largest group of muscle diseases for which a potential treatment exists. They play a significant role among rheumatological and neurological patients, both in children and adults. The management of these patients often requires an interdisciplinary approach. These conditions are a very heterogeneous group of diseases, which were traditionally catogerised in dermatomyositis, polymyositis, and later also in sporadic inclusion body myositis. In the past few years, substantial insight has been gained regarding pathogenesis, autoantibodies, involvement of various organs, etc., which means that this simplified concept is no longer tenable. Based on this new insight, new classification systems have been developed and refined. Today there are 6 different entities: dermatomyositis (DM), polymyositis (PM), autoimmune-mediated necrotizing myositis (ANM), anti-synthetase-syndrome-associated myositis (ASS-associated myositis), nonspecific myositis (NSM) and sporadic inclusion body myositis (sIBM) ([Stenzel 2015], [Hoogendijk 2004]). Except for sIBM, these groups are also seen in children, albeit with a differently weighted distribution pattern of the individual sub-entities compared with adults ([Rider 2013]). The distinction between these categories is fundamental to prognosis and treatment response. Therefore, it appears to be very important to correctly identify, name and distinguish them from other diseases with clinical similarities. This review aims to summarise the current knowledge about the aforementioned entities in the light of clinico-pathological aspects and to illustrate the histopathology of these diseases.

Schlüsselwörter

Myopathie - Entzündung - Differenzialdiagnose

Key words

myopathy - inflammation - differential diagnosis
 

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