Janus kinase inhibitors are of limited use in refractory JIA-associated uveitis: Retrospective data from a tertiary uveitis center

Background Juvenile idiopathic arthritis-associated uveitis (JIAU) frequently leads a chronic course, and ocular complications are frequent. Janus kinase inhibitors (JAKi) might represent a promising new therapeutic option in therapy-refractory cases. Methods Retrospective monocentric analysis of 20 children with chronic anterior JIAU treated with either tofacitinib (n = 18), baricitinib (n = 1) or upadacitinib (n = 1). Results All patients had received methotrexate prior to baseline, and at least two biologics had been ineffective in 18/20 patients. With one of the JAKi, eight patients achieved uveitis inactivity (anterior chamber cell count < 0.5+) at any time during follow-up (mean 9.2 ± 3.3 months), but uveitis relapsed in 6 of them subsequently. In 7 patients, treatment was terminated after ≤ 1 year due to inadequate response of ocular inflammation. During follow-up, new uveitis-related complications occurred in 7 patients (9 eyes), the most frequent were macular edema (8 eyes of 7 patients), and ocular hypertension (5 eyes of 3 patients). Reduction of concomitant systemic medication was possible in 3/14 patients. Any reduction of topical steroids during follow-up was possible in 24/30 eyes, and 23/30 eyes received a lower dose at the last follow-up than they did at study inclusion. Conclusions Our data show limited efficiency of JAKi treatment in patients with chronic DMARD-refractory JIAU. The number of patients in whom uveitis quiescence could be achieved was low, as was the possibility for reduction of both concomitant systemic medication and topical steroids, and the relapse rate during the course of treatment was high.