{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T11:47:54Z","timestamp":1788176874935,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,4,18]],"date-time":"2025-04-18T00:00:00Z","timestamp":1744934400000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Recent studies have revealed a shared genetic background and pathogenesis between Beh\u00e7et's syndrome (BS) and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. This study suggests genetic similarities between recurrent aphthous stomatitis, PFAPA syndrome, and BS and proposes the concept of Beh\u00e7et's spectrum disorders. An 18\u2010year\u2010old female with a history of PFAPA syndrome who had experienced repeated attacks until puberty visited our hospital complaining of oral ulcers, arthralgia, rash, and abdominal pain that had persisted for 2\u2009weeks. Colonoscopy revealed a deep ulcer in the ileocecal region. Pathological findings showed no evidence suggestive of viral infection, malignancy, or vasculitis, and only nonspecific inflammatory cell infiltration. After ruling out various autoinflammatory diseases through genetic testing, the patient was diagnosed with Beh\u00e7et's spectrum disorder. The patient was refractory to adalimumab and achieved remission after upadacitinib therapy. Genetic similarities have been reported between PFAPA syndrome and BS, involving genes such as IL\u201010 and IL\u201012A. Since the IL\u201010, IL\u201012, and IL\u201023 pathways can be blocked by JAK inhibitors, JAK inhibition therapy may be a potentially effective treatment option for Beh\u00e7et's syndrome in a patient with a history of PFAPA syndrome.<\/jats:p>","DOI":"10.1111\/1756-185x.70231","type":"journal-article","created":{"date-parts":[[2025,4,19]],"date-time":"2025-04-19T01:12:06Z","timestamp":1745025126000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Case Report: Successful Remission With Upadacitinib in Beh\u00e7et's Syndrome Following a History of\n                    <scp>PFAPA<\/scp>\n                    Syndrome"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9810-7644","authenticated-orcid":false,"given":"Hideki","family":"Oka","sequence":"first","affiliation":[{"name":"Department of Rheumatology Kobe City Medical Center General Hospital  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4980-2664","authenticated-orcid":false,"given":"Shuji","family":"Sumitomo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Kobe City Medical Center General Hospital  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisuke","family":"Yamashita","sequence":"additional","affiliation":[{"name":"Department of Pathology Kobe City Medical Center General Hospital  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2927-9159","authenticated-orcid":false,"given":"Koichiro","family":"Ohmura","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Kobe City Medical Center General Hospital  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,4,18]]},"reference":[{"key":"e_1_2_1_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2305712"},{"key":"e_1_2_1_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/gene.2014.64"},{"key":"e_1_2_1_9_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2020-217307"},{"key":"e_1_2_1_9_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.981502"},{"key":"e_1_2_1_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80285-8"},{"key":"e_1_2_1_9_7_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2002051117"},{"key":"e_1_2_1_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70321-5"},{"key":"e_1_2_1_9_9_1","first-page":"59","article-title":"Recent Research Into Beh\u00e7et's Disease in Japan","volume":"10","author":"Mizushima Y.","year":"1988","journal-title":"International Journal of Tissue Reactions"},{"key":"e_1_2_1_9_10_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100028\u20101103481"},{"key":"e_1_2_1_9_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis\u20102018\u2010213225"},{"key":"e_1_2_1_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/jgh.15754"},{"key":"e_1_2_1_9_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-40824-y"},{"key":"e_1_2_1_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2008.08.005"}],"container-title":["International Journal of Rheumatic Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1756-185X.70231","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T11:28:41Z","timestamp":1765366121000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1756-185X.70231"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4]]},"references-count":13,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,4]]}},"alternative-id":["10.1111\/1756-185X.70231"],"URL":"https:\/\/doi.org\/10.1111\/1756-185x.70231","archive":["Portico"],"relation":{},"ISSN":["1756-1841","1756-185X"],"issn-type":[{"value":"1756-1841","type":"print"},{"value":"1756-185X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4]]},"assertion":[{"value":"2024-10-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-12","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70231"}}