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Großgefäßvaskulitis

Large vessel vasculitis

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Zusammenfassung

Die Riesenzellarteriitis (RZA) und die Takayasu-Arteriitis (TA) werden unter dem Begriff „Großgefäßvaskulitis“ (GGV) zusammengefasst. Bei den GGV handelt es sich um Autoimmunerkrankungen mit granulomatöser Gefäßentzündung der mittelgroßen und großen Arterien. GGV gehen mit Zeichen einer systemischen Entzündungsreaktion einher wie BSG- und CRP-Erhöhung, Abgeschlagenheit, subfebrilen Temperaturen und Gewichtsverlust. Spezifischere Symptome sind vor allem Kopfschmerzen und visuelle Symptome bei der RZA, Arm- oder Bein-Claudicatio, renale Hypertension oder pektanginöse Beschwerden bei der TA. Die Diagnostik stützt sich hauptsächlich auf bildgebende Methoden zum Nachweis der entzündlichen Gefäßveränderungen sowie ggf. eine Temporalarterienbiopsie bei der RZA. Glukokortikoide stellen die primäre Therapie dar. Methotrexat scheint einen Glukokortikoid-sparenden Effekt zu haben, während die Datenlage für andere Immunsuppressiva wie Azathioprin, Tocilizumab oder Cyclophosphamid beschränkt ist. Bei der TA sind darüber hinaus ggf. Revaskularisationsmaßnahmen notwendig.

Abstract

Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the two diseases characterized as large vessel vasculitis (LVV) and are autoimmune diseases with granulomatous inflammation that affect medium and large sized arteries. These diseases are accompanied by symptoms of systemic inflammatory reactions typically including fatigue, weight loss and low grade fever as well as elevation of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. More specific symptoms include headache and visual symptoms for GCA and arm or leg claudication, renal hypertension and angina pectoris for TA. Imaging studies to demonstrate inflammatory vascular wall lesions and biopsy of the temporal artery for GCA are the most relevant diagnostic procedures. Treatment relies mainly on glucocorticoids. Methotrexate seems to have a moderate glucocorticoid-sparing effect but evidence for other immunosuppressants, including azathioprine, tocilizumab and cyclophosphamide is limited. Revascularization methods might also be required in TA.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. S. Weigand gibt an, dass kein Interessenkonflikt besteht. M. Fleck: Honorare für Vortrags- und Beratungstätigkeit von der Fa. Roche. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Klinik und Poliklinik für Rheumatologie/klinische Immunologie, Asklepios Klinikum Bad Abbach, Kaiser-Karl V,-Allee 3, 93077, Bad Abbach, Deutschland

    S. Weigand & M. Fleck

  2. Klinik und Poliklinik für Innere Medizin I, Universitätsklinikum Regensburg, Regensburg, Deutschland

    M. Fleck

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Correspondence to S. Weigand.

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Weigand, S., Fleck, M. Großgefäßvaskulitis. Z. Rheumatol. 73, 447–457 (2014). https://doi.org/10.1007/s00393-014-1416-x

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  • DOI: https://doi.org/10.1007/s00393-014-1416-x

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