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Skin and joint findings typically emerge by age 2\u2009years, with ocular involvement appearing around age 4\u2009years. Early biologic intervention, particularly with anti\u2010TNF\u2010\u03b1 therapies, can prevent severe complications like joint destruction and blindness. Systemic corticosteroids serve as bridging therapy, while methotrexate and thalidomide may help but are often insufficient as monotherapy. IL\u20101 and IL\u20106 inhibitors, as well as tofacitinib, are options for refractory cases.<\/jats:p>","DOI":"10.1111\/pde.16001","type":"journal-article","created":{"date-parts":[[2025,6,2]],"date-time":"2025-06-02T20:03:51Z","timestamp":1748894631000},"page":"728-734","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Blau Syndrome (Juvenile Systemic Granulomatosis): State\u2010Of\u2010The\u2010Art Review"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-2217-0161","authenticated-orcid":false,"given":"Ou Jia (Emilie)","family":"Wang","sequence":"first","affiliation":[{"name":"Faculty of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianna","family":"Jacob","sequence":"additional","affiliation":[{"name":"Department Dermatology and Skin Science University of British Columbia  Vancouver 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