Uveitis in Juvenile Idiopathic Arthritis: 18-Year Outcome in the Population-based Nordic Cohort Study

Veronika Rypdal,Suvi Peltoniemi,Lillemor Berntson,Kristiina Aalto,Mia Glerup,Anders Fasth,Troels Herlin,Peter Toftedal,Nils Thomas Songstad,Regitze Bangsgaard,Terje Christoffersen,Susan Nielsen,Ellen Nordal,Sanna Leinonen,Geir Bertelsen,Maria Ekelund,Marite Rygg,Ellen Dalen Arnstad ,Rasmus Gaardskær Nielsen ,Marek Žák

doi:10.1016/j.ophtha.2020.08.024

Abstract

To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA. A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden. Patients with onset of JIA between January 1997 and June 2000 were prospectively followed for 18 years. Pediatric rheumatologists and ophthalmologists collected clinical and laboratory data. Cumulative incidence of uveitis and clinical characteristics, JIA and uveitis disease activity, ocular complications, visual outcome, and risk factors associated with the development of uveitis-related complications. Uveitis developed in 96 (22.1%) of 434 patients with JIA. In 12 patients (2.8%), uveitis was diagnosed between 8 and 18 years of follow-up. Systemic immunosuppressive medication was more common among patients with uveitis (47/96 [49.0%]) compared with patients without uveitis (78/338 [23.1%]). Active uveitis was present in 19 of 78 patients (24.4%) at the 18-year visit. Ocular complications occurred in 31 of 80 patients (38.8%). Short duration between the onset of JIA and the diagnosis of uveitis was a risk factor for developing ocular complications (odds ratio [OR], 1.4; 95% confidence interval [CI], 1.1-1.8). Patients with a diagnosis of uveitis before the onset of JIA all developed cataract and had an OR for development of glaucoma of 31.5 (95% CI, 3.6-274). Presence of antinuclear antibodies (ANAs) was also a risk factor for developing 1 or more ocular complications (OR, 3.0; 95% CI, 1.2-7.7). Decreased visual acuity (VA) <6/12 was found in 12 of 135 eyes (8.9%) with uveitis, and 4 of 80 patients (5.0%) with JIA-U had binocular decreased VA <6/12. Our results suggest that uveitis screening should start immediately when the diagnosis of JIA is suspected or confirmed and be continued for more than 8 years after the diagnosis of JIA. Timely systemic immunosuppressive treatment in patients with a high risk of developing ocular complications must be considered early in the disease course to gain rapid control of ocular inflammation.

Highlights

To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA)
Our study describes the long-term clinical outcome in JIAassociated uveitis (JIA-U) in terms of cumulative incidence, the use of immunosuppressive treatment, visual outcome, ocular complications, and risk factors associated with the development of ocular complications
All patients who were diagnosed with uveitis before JIA and 9 of the 12 patients diagnosed with uveitis between the 8-year and 18-year followups were among the 80 patients with JIA-U examined by the ophthalmologist

Summary

Introduction

To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Design: Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIAassociated uveitis (JIA-U) 18 years after the onset of JIA. There are few long-term prospective studies on uveitis in JIA.[4,14,15] Studies have shown that the majority of patients with JIA-U develop uveitis within the first 4 years after the onset of JIA5,16,17 and that high-grade uveitis and active uveal inflammation are associated with higher complication rates.[11,13,18,19] At diagnosis, ocular complications are seen in up to 21% to 76% of eyes with JIA-U,11,13 and further complications are reported to occur during the course with a complication rate of 0.33 per eye per year.[18] our knowledge is limited regarding long-term complications and complications in patients who develop uveitis late in the course of JIA.[14,16]. Previous studies have reported that female gender, young age at onset of JIA, oligoarticular JIA, and the presence of antinuclear antibodies (ANAs) and human leukocyte antigen (HLA)-B27 are risk factors for the development of uveitis in JIA.[3,5,20] Reported risk factors for ocular complications in established uveitis are male gender, the onset of uveitis before arthritis, short interval between the onsets of JIA and uveitis, and the presence of ocular complications early in the disease course.[6,21] there are inconsistencies between studies concerning risk factors of ocular complications in JIA-U

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