Riesenzellarteriitis und Takayasu Arteriitis: Klinische Aspekte, Diagnostik und Therapie

 

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Zusammenfassung

Die Riesenzellarteriitis (RZA) ist die häufigste systemische Vaskulitis im Erwachsenenalter, und betrifft die großen und mittelgroßen Arterien. Die Takayasu Arteriitis (TA) ist auch eine Vaskulitis der großen Gefäße und tritt Im Gegensatz zur RZA typischerweise vor dem 40. Lebensjahr auf. Sie ist die dritthäufigste Vaskulitis im Kindesalter. Beide Großgefäßvaskulitiden können zu gefürchteten ischämischen Komplikationen im Bereich der Extremitäten oder der viszeralen Organe führen. Aus diesem Grunde ist eine frühzeitige Diagnosestellung von großer Bedeutung. Die häufigste Komplikation der RZA ist die anteriore ischämische Optikusneuropathie, welche unbehandelt zur Erblindung führen kann. In jedem Fall soll die Diagnose histologisch oder sonografisch bestätigt werden. Die Temporalarterienbiopsie oder Bildgebung sollte so früh wie möglich stattfinden, den Therapiebeginn bei dringendem klinischem Verdacht jedoch nicht verzögern. Desweiteren haben bei der TA die Angiografie und bei beiden Großgefäß­vaskulitiden im Hinblick auf die Erstdiagnostik und die Verlaufsbeobachtung die Magnetresonanzangiografie, die CT-Angiografie, die Positronenemis­sionstomografie und nicht zuletzt der Ultraschall einen hohen Stellenwert. Sowohl die RZA als auch die TA zeichnen sich durch ein Ansprechen auf Kortikosteroide aus. Die Rolle von Immunsuppressiva ist noch nicht vollständig geklärt. Bei der Behandlung der RZA liegen derzeit zu Methotrexat als Kortikosteroid sparende Therapie die meisten Daten vor. Im Rahmen der Takayasu Arteriitis haben die TNFalpha-Blocker eine Bedeutung.

Abstract

Giant cell arteritis (GCA) is the most common systemic vasculitis and affects large and medium-sized vessels. Takayasu arteritis (TA) is the second vasculitis of the large-sized vessels and affects young adults under 40 years of age. TA is the third most common vasculitis in children. Both vasculitides can cause ischaemic complications of extremities and visceral organs. The most common complication of GCA is ischaemia of the optic nerve causing irreversible blindness. Besides, dissection of aortic aneurysms, cerebral ischaemia, ischaemia of limbs or organs as well as tongue necrosis may occur. Diagnosis shoud be confirmed by biopsy or sonography but diagnostic procedures shoud not delay therapy. In addition, angiography is important as a diagnostic procedure in TA. In both large-sized vasculitides magnetic resonance angiography, computed tomography angiography, positron emission tomography and last but not least colour Dopler ultrasonography enable early diagnosis and the monitoring of responses to treatment. Cortico­steroids are the therapy of choice in the treatment of GCA and TA. Disease-modifying drugs particularly methotrexate have a corticosteroid sparing effect in GCA. In contrast, TNF-blocking agents are effective in the treatment of TA.

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