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Klinisch zeichnet sich das VEXAS-Syndrom durch ein Mischbild aus rheumatologischer Erkrankung mit separaten h\u00e4matologischen Pathologien aus, wobei besonders h\u00e4ufig eine Polychondritis und fast immer eine makrozyt\u00e4re An\u00e4mie auftreten. Anhand dieser Kasuistik werden die diagnostischen Kernpunkte zur Erkennung des VEXAS-Syndroms demonstriert.<\/jats:p>","DOI":"10.1007\/s00393-023-01318-5","type":"journal-article","created":{"date-parts":[[2023,2,3]],"date-time":"2023-02-03T07:02:42Z","timestamp":1675407762000},"page":"229-233","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Makrozyt\u00e4re An\u00e4mie und Polychondritis: VEXAS-Syndrom","Macrocytic anemia and polychondritis: VEXAS syndrome"],"prefix":"10.1007","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5575-0787","authenticated-orcid":false,"given":"Markus","family":"Zeisbrich","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Viktoria","family":"Schindler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e1t\u00e9","family":"Krausz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Proietti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pavla","family":"Mrovecova","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reinhard E.","family":"Voll","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cornelia","family":"Glaser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabian","family":"R\u00f6ther","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Warnatz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nils","family":"Venhoff","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,2,3]]},"reference":[{"issue":"27","key":"1318_CR1","doi-asserted-by":"publisher","first-page":"2628","DOI":"10.1056\/NEJMoa2026834","volume":"383","author":"DB Beck","year":"2020","unstructured":"Beck\u00a0DB, Ferrada\u00a0MA, Sikora\u00a0KA, Ombrello\u00a0AK, Collins\u00a0JC, Pei\u00a0W et al (2020) Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease. 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