Successful Remission with Upadacitinib in Two Patients with Anti-TNF-Refractory Macular Edema Associated with Behçet’s Uveitis

ABSTRACTPurpose Behçet's syndrome (BS) is a chronic inflammatory disease affecting the small and large vessels of the venous and arterial systems and is characterized by recurrent oral and genital ulcers. Uveitis represents the most typical ocular manifestation and completes the triple symptom complex originally described. Recognized treatments for Behçet's uveitis (BU) include systemic glucocorticoids and immunosuppressive agents. No study has reported on the use of upadacitinib for BS with panuveitis. Herein, we report the use of upadacitinib in two patients with BU suffering from macular edema and persistent inflammation, which was refractory to systemic glucocorticoids and immunosuppressive agents.Methods We retrospectively followed-up two cases, including an adolescent girl and a man in his thirties, with a 2- and 10-year history of BS, respectively.Results Upadacitinib successfully treated BU, leading to improved visual acuity, controlled intraocular inflammation, and the disappearance of macular edema in both patients. The patients in this study were either recalcitrant to or intolerant to conventional therapy and adalimumab. Only the female patient revealed a mildly abnormal blood picture and slight transaminitis after 6 months of upadacitinib administration. However, no serious adverse events were reported in either of the two patients during follow-up.Conclusion Upadacitinib can be considered an important future option for managing recurrent and recalcitrant cases of BU, especially in those with chronic ocular inflammation and macular edema, which are refractory to conventional therapies.KEYWORDS: Behçet's diseasecystoid macular edemaJAK inhibitorupadacitinibuveitis Disclosure statementNo potential conflict of interest was reported by the author(s).Ethics declarationsI confirm that all the research meets ethical guidelines and adheres to the legal requirements of the study country.Additional informationFundingThis work was supported by the National Outstanding Youth Science Fund Project of China (No. 82122016).