{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T06:58:48Z","timestamp":1769151528227,"version":"3.49.0"},"reference-count":27,"publisher":"Georg Thieme Verlag KG","issue":"09","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Monbl Augenheilkd"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>\n          Hintergrund Die okul\u00e4re Beteiligung bei vernarbendem Schleimhautpemphigoid (SHP) ist mit einer Pr\u00e4valenz von 25\u00a0F\u00e4llen je 1\u2009Mio. Einwohner und damit ca. 2100 Patienten in ganz\n          Deutschland selten. Die Diagnosestellung kann \u2013 besonders in Abwesenheit anderer Beteiligungen \u2013 schwierig und die Therapie komplex und langwierig sein. Nicht selten kommen Immunsuppressiva\n          zum Einsatz. Aufgrund der Komplexit\u00e4t von Diagnose und Therapie sind SHP-Patienten meist an entsprechend spezialisierte Zentren angebunden. Ziel dieses Projektes war die Erfassung der\n          aktuellen augen\u00e4rztlichen Versorgungssituation von Patienten mit SHP in Deutschland.<\/jats:p><jats:p>\n          Methoden Eine papierbasierte Umfrage wurde konzipiert und im April 2020 an alle Universit\u00e4tsaugenkliniken und weitere potenzielle Zentren versandt. Gefragt wurde nach dem Bestehen\n          einer spezialisierten Sprechstunde, der j\u00e4hrlichen Gesamtzahl der betreuten Patienten, der j\u00e4hrlichen Anzahl von neu diagnostizierten Patienten, den klinischen Kooperationspartnern in\n          Diagnostik und Therapie sowie nach der angewendeten lokalen und systemischen Therapie.<\/jats:p><jats:p>\n          Ergebnisse Von insgesamt 44 angeschrieben Kliniken erfolgten 28 (64%) vollst\u00e4ndige R\u00fcckmeldungen. Im Mittel werden in den Kliniken 27\u2009\u00b1\u200942 (0\u200a\u2013\u200a200) Patienten betreut und j\u00e4hrlich\n          pro Zentrum 3,6\u2009\u00b1\u20092,2 (0\u200a\u2013\u200a10) neue F\u00e4lle diagnostiziert. Dies entspricht einer Gesamtpatientenzahl von 741 Patienten. Lediglich 9 (32%) der antwortenden Kliniken bieten eine spezialisierte\n          SHP-Sprechstunde an. 93% der Zentren kooperieren mit der lokalen Klinik f\u00fcr Dermatologie. 79% f\u00fchren die serologische und histologische Diagnostik intern durch. Etwa die H\u00e4lfte der Zentren\n          (n\u2009=\u200916) wendet ein standardisiertes Therapieschema an. Systemisch werden Glukokortikoide (66,7%) am h\u00e4ufigsten verwendet, gefolgt von Mycophenolatmofetil und Dapson (57,1%), Rituximab\n          (33,3%), Azathioprin und Cyclophosphamid (28,6%) sowie Methotrexat (19,0%). Am seltensten werden i.\u200av. Immunoglobuline eingesetzt (14,3%).<\/jats:p><jats:p>\n          Schlussfolgerung Mit dieser Umfrage unter deutschen augen\u00e4rztlichen Zentren wurden Daten von etwa einem Drittel der gesch\u00e4tzten Gesamtzahl aller in Deutschland an einem SHP\n          erkrankten Menschen erhoben. Dabei handelt es sich vermutlich ausschlie\u00dflich um Patienten mit mindestens einer okul\u00e4ren Beteiligung. Aktuell wird eine augen\u00e4rztliche SHP-Registerstudie\n          etabliert, um die Epidemiologie und Versorgungssituation besser zu erfassen und langfristig zu verbessern.<\/jats:p>","DOI":"10.1055\/a-1720-1819","type":"journal-article","created":{"date-parts":[[2022,5,24]],"date-time":"2022-05-24T23:18:16Z","timestamp":1653434296000},"page":"1077-1083","source":"Crossref","is-referenced-by-count":7,"title":["Zur Versorgungssituation und konservativen Therapie des okul\u00e4ren vernarbenden Schleimhautpemphigoids in Deutschland"],"prefix":"10.1055","volume":"240","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7476-2724","authenticated-orcid":false,"given":"R\u00e9mi","family":"Ya\u00efci","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Augenheilkunde, Universit\u00e4tsklinikum D\u00fcsseldorf, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mathias","family":"Roth","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr 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