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Wegweisende Befunde sind rezidivierende Thrombosen des ven\u00f6sen Systems und oberfl\u00e4chliche Thrombophlebitiden in Kombination mit beidseitigen Pulmonalarterienaneurysmen (PAA). Die Pulmonalisangiographie mittels Computertomographie ist von entscheidender diagnostischer Bedeutung, um die (entz\u00fcndliche) Beteiligung der Pulmonalarterien darzustellen. Die Therapie des HSS orientiert sich an den Empfehlungen der European Alliance of Associations for Rheumatology (EULAR) f\u00fcr das BS und sieht prim\u00e4r eine Immunsuppression mit Cyclophosphamid und Glukokortikoiden vor. Neben einer medikament\u00f6sen Therapie sollte eine interventionelle Versorgung der PAA evaluiert werden. Eine spontane PAA-Ruptur muss auch bei Remission der Erkrankung und\/oder deutlicher Regredienz des PAA-Durchmessers aufgrund einer fragilen Gef\u00e4\u00dfarchitektur bedacht werden.<\/jats:p>","DOI":"10.1007\/s00393-023-01371-0","type":"journal-article","created":{"date-parts":[[2023,6,6]],"date-time":"2023-06-06T19:32:08Z","timestamp":1686079928000},"page":"327-333","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Hughes-Stovin-Syndrom: eine lebensbedrohliche Manifestation des Beh\u00e7et-Syndroms","Hughes-Stovin syndrome: a\u00a0life-threatening manifestation of Beh\u00e7et\u2019s syndrome"],"prefix":"10.1007","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8394-969X","authenticated-orcid":false,"given":"Nikolas","family":"Ruffer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Krusche","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konstanze","family":"Holl-Ulrich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabian","family":"L\u00f6tscher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ina","family":"K\u00f6tter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,6,6]]},"reference":[{"issue":"1","key":"1371_CR1","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1016\/s0007-0971(59)80106-6","volume":"53","author":"JP Hughes","year":"1959","unstructured":"Hughes\u00a0JP, Stovin\u00a0PG (1959) Segmental pulmonary artery aneurysms with peripheral venous thrombosis. 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