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Vaskulitis

Neue Nomenklatur der Chapel-Hill-Konsensuskonferenz 2012

Vasculitis

New nomenclature of the Chapel Hill consensus conference 2012

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Zusammenfassung

In den letzten Jahren konnten für einige Vaskulitisformen wesentliche ätiopathogenetische Mechanismen geklärt und unterschiedliche Verlaufsformen abgegrenzt werden. Die Nomenklatur der revidierten Chapel-Hill-Konsensuskonferenz (CHCC) 2012 für Vaskulitiden berücksichtigt nunmehr diese wesentlichen ätiopathogenetischen Zusammenhänge zusätzlich zu den deskriptiven Prinzipien wie Gefäßgröße und Entzündungstyp. ANCA-assoziierte Vaskulitiden wurden erstmals als separate Gruppe den Immunkomplexkleingefäßvaskulitiden gegenübergestellt. Wo ein Konsens zu systematischen Namen erzielt wurde, sind Eponyme hierdurch ersetzt worden, so bei der Wegener-Granulomatose, jetzt Granulomatose mit Polyangiitis, und beim Churg-Strauss-Syndrom, jetzt eosinophile Granulomatose mit Polyangiitis. Daneben sind klinisch wichtige, teilweise weniger beachtete Vaskulitisformen erstmals in die Nomenklatur integriert worden. Der Beitrag stellt die Neuerungen vor und fasst wichtige aktuelle Arbeiten zu Klinik und Morphologie der Vaskulitiden zusammen.

Abstract

Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener’s granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. K. Holl-Ulrich gibt an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren. Diese Arbeit wurde unterstützt durch Mittel aus der DFG-geförderten klinischen Forschergruppe 170 (Teilprojekt 4): „Frühpathogenese der Wegener’schen Granulomatose“.

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Authors and Affiliations

  1. Konsultations- und Referenzzentrum für Vaskulitisdiagnostik, Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    K. Holl-Ulrich

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  1. K. Holl-Ulrich
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Correspondence to K. Holl-Ulrich.

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__ ____

Dieser Beitrag ist eine aktualisierte und neu bearbeitete Fassung des Beitrags: K. Holl-Ulrich (2013) Vaskulitis. Der Pathologe 34:569–581. doi 10.1007/s00292-013-1796-8.

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Holl-Ulrich, K. Vaskulitis. Z. Rheumatol. 73, 823–835 (2014). https://doi.org/10.1007/s00393-014-1477-x

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  • DOI: https://doi.org/10.1007/s00393-014-1477-x

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