Zusammenfassung
Großgefäßvaskulitiden manifestieren sich an der Aorta und ihren Ästen erster und zweiter Ordnung durch umschriebene Entzündungen der Gefäßwand, in denen regelhaft Riesenzellen nachweisbar sind. In der Folge können Stenosen und Verschlüsse ebenso entstehen wie aneurysmatische Aufweitungen. Fortschritte in der bildgebenden Diagnostik (Farbduplex, MRT/MRA und PET) lassen die Diagnose bei der Takayasu-Arteriitis früher als bisher stellen und decken bei der Arteriitis temporalis (Riesenzellarteriitis, RZA) den Befall auch größerer Arterien auf. Zunehmend häufig wird bei jüngeren, überwiegend weiblichen Patienten mit RZA eine weitgehend extrakraniale Variante diagnostiziert, die vor allem die A. subclavia, teilweise auch die Aorta befällt. Letztere kann auch isoliert betroffen sein. Die Initialbehandlung erfolgt mit Steroiden, als weiteres Immunsuppressivum hat sich bei der RZA nur Methotrexat bewährt. Die Krankheitsaktivität wird anhand serologischer Entzündungsparameter, aber auch anhand der Gefäßmorphologie überwacht.
Abstract
Large vessel vasculitis is characterized by local inflammation of the vessel wall of the aorta and its first and second degree branches. Giant cells are found regularly. The process leads to stenoses and occlusions as well as to aneurysmatic dilatation. Recent progress in vascular imaging (colour Doppler ultrasound, MRI and PET) allows to detect Takayasu arteritis at an earlier stage and to reveal the involvement of larger arteries in temporal arteritis (giant cell arteritis, GCA). With increasing frequency, a subtype of predominantly female and less aged GCA patients without cranial symptoms is noticed in whom obstructions are prevailing in the proximal arm vessels or in the aorta. The latter may be affected exclusively. Initial therapy is based on corticosteroids. In GCA, methotrexate hitherto is the only other immunosuppressive drug that has proved to be helpful. Disease activity has to be controlled both by laboratory parameters and vascular imaging.
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Caspary, L., Schellong, S. Vaskulitiden der großen Gefäße. Internist 50, 958–963 (2009). https://doi.org/10.1007/s00108-009-2363-5
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DOI: https://doi.org/10.1007/s00108-009-2363-5