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 follows a chronic and complicated course. Janus kinase inhibitors (JAKi) represent a promising new therapeutic option. Methods Retrospective monocentric analysis of 20 children with chronic anterior JIAU treated with tofacitinib ( n = 18), baricitinib ( n = 1) or upadacitinib ( n = 1). Outcome measures: uveitis or arthritis inactivity at any time during follow-up, recurrences after achieving inactivity, sparing of medication, occurrence of new ocular complications, resolution of preexisting macular edema. Results All patients had received methotrexate prior to baseline, and at least two biologics had been ineffective in 18/20 patients. Whilst receiving JAKi medication, uveitis was inactive in eight patients (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 baseline. Conclusions Our data show limited effectiveness of JAKi treatment in patients with chronic DMARD-refractory JIAU. The number of patients in whom uveitis quiescence could be observed was low, as was the possibility for reduction of concomitant medication, and the relapse rate was high. Clinical trial number Not applicable.