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DOI: 10.1055/s-2005-858625
© Georg Thieme Verlag KG Stuttgart · New York
CAR-Syndrom (carcinoma-associated retinopathy syndrome) assoziiert mit einem Adenokarzinom der Zervix
CAR Syndrome (Carcinoma-Associated Retinopathy Syndrome) Associated with an Adenocarcinoma of the CervixPublication History
Eingegangen: 23.12.2004
Angenommen: 7.7.2005
Publication Date:
20 September 2005 (online)
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Zusammenfassung
Hintergrund: Das CAR-Syndrom ist eine seltene paraneoplastische Erkrankung. Die häufigste Assoziation besteht zum kleinzelligen Bronchialkarzinom. In der Pathogenese spielen Antikörper eine Rolle, die gegen die Retina und auch andere okuläre Strukturen gerichtet sind. Diese induzieren die Apoptose in den retinalen Photorezeptorzellen. Fallbeschreibung: Wir berichten über eine 68-jährige Patientin, die sich im Februar 2003 wegen Sehstörungen mit progredienter Visusverschlechterung und Gesichtsfeldeinschränkung vorstellte, welche anamnestisch erstmalig im Jahre 1998 aufgetreten waren. Ergebnisse: Bei der Erstuntersuchung fanden sich beidseits ein Visus von 0,1 und konzentrische Gesichtsfelddefekte. Das Elektroretinogramm zeigte skotopisch und photopisch reduzierte Amplituden. In der Fluoreszenzangiographie fanden sich beidseits eine Uveitis intermedia, eine Engstellung der Gefäße, eine Optikusatrophie und punktförmige Pigmentepitheldefekte. Da anamnestisch ein Zustand nach Operation eines Adenokarzinoms der Zervix im Juni 2002 bestand, wurde die Verdachtsdiagnose CAR-Syndrom gestellt. Die Serumdiagnostik ergab im Western Blot einen positiven Befund bezüglich verschiedener retinaler Autoantikörper. Anhand der Befunde konnte die Diagnose des CAR-Syndroms gesichert werden. Eine Therapie wurde mittels hoch dosierter systemischer Kortikosteroide durchgeführt. Hierunter zeigte sich im weiteren Verlauf ein stabiler Befund. Schlussfolgerungen: Bei einer unklaren, progredienten Visusverschlechterung mit einer konzentrischen Gesichtsfeldeinengung und einem pathologischen Elektroretinogramm sowie vorliegenden Zeichen einer Uveitis ist stets differenzialdiagnostisch ein CAR-Syndrom auszuschließen. Es ist zu bedenken, dass die ophthalmologischen Symptome deutlich vor der Erstdiagnose des Karzinoms auftreten können.
Abstract
Background: CAR is a rare paraneoplastic syndrome. It is most commonly associated with small-cell carcinoma of the lung. Specific autoantibodies exist not only against the retina but also against other ocular structures. They induce apoptotic death of retinal photoreceptor cells. Case report: We report about a 68-year-old patient who presented in February 2003 with progressive visual dysfunction including visual acuity loss and visual field defects of unknown origin, which first manifested in the year 1998. Results: We found a visual acuity of 0.1 in both eyes and concentric visual field defects. The scotopic and photopic electroretinogram was markedly reduced. Uveitis intermedia in both eyes, narrowing of the vessels, especially of the arterioles, optic atrophy and window defects in the retinal pigment epithelium were observed on fluorescein angiography. Due to the patient’s report about a resection of an adenocarcinoma of the cervix in June 2002, she was diagnosed as having CAR syndrome. Serologic screening by Western blot analysis revealed different specific autoantibodies against retinal proteins. The patient was treated with corticosteroid pulse therapy. The follow-up showed stable findings. Conclusions: In patients with progressive visual loss, concentric visual field defects and pathological electroretinogram as well as evidence of uveitis, a CAR syndrome has to be ruled out. One has to take into consideration that visual dysfunction can appear before the primary cancer is diagnosed.
Schlüsselwörter
CAR-Syndrom - Uveitis - retinale Autoantikörper
Key words
CAR syndrome - uveitis - autoantibodies against the retina
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Dr. med. S. Seles
Universitätsaugenklinik Ulm
Prittwitzstraße 43
89075 Ulm
Email: sseles@web.de