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  • Review Article
  • Published:

Uveitis associated with juvenile idiopathic arthritis

Nature Reviews Rheumatology volume 11, pages 338–348 (2015)Cite this article

Subjects

Key Points

  • Uveitis affects 11–30% of children with juvenile idiopathic arthritis (JIA)

  • Chronic anterior uveitis is usually asymptomatic initially but can lead to visually disabling complications; therefore, screening is essential

  • Initial treatment is with topical corticosteroids but evidence supports the early introduction of systemic immunosuppressive drugs, such as methotrexate, as steroid-sparing agents

  • Prospective controlled studies of biologic therapies, including adalimumab and tocilizumab, are underway or planned

  • Future research to investigate the pathogenesis of JIA-associated uveitis and identify novel biomarkers could enable earlier diagnosis and more-effective treatment

Abstract

Uveitis is a potentially sight-threatening complication of juvenile idiopathic arthritis (JIA). JIA-associated uveitis is recognized to have an autoimmune aetiology characterized by activation of CD4+ T cells, but the underlying mechanisms might overlap with those of autoinflammatory conditions involving activation of innate immunity. As no animal model recapitulates all the features of JIA-associated uveitis, questions remain regarding its pathogenesis. The most common form of JIA-associated uveitis is chronic anterior uveitis, which is usually asymptomatic initially. Effective screening is, therefore, essential to detect early disease and commence treatment before the development of visually disabling complications, such as cataracts, glaucoma, band keratopathy and cystoid macular oedema. Complications can result from uncontrolled intraocular inflammation as well as from its treatment, particularly prolonged use of high-dose topical corticosteroids. Accumulating evidence supports the early introduction of systemic immunosuppressive drugs, such as methotrexate, as steroid-sparing agents. Prospective randomized controlled trials of TNF inhibitors and other biologic therapies are underway or planned. Future research should aim to identify biomarkers to predict which children are at high risk of developing JIA-associated uveitis or have a poor prognosis. Such biomarkers could help to ensure that patients receive earlier interventions and more-potent therapy, with the ultimate aim of reducing loss of vision and ocular morbidity.

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Figure 1: Cross-section of eye showing areas affected in anterior, intermediate and posterior uveitis.
Figure 2: Immunopathogenesis of uveitis.

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Acknowledgements

A.D.D.'s research work was partly supported by the National Institute for Health Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital National Health Service (NHS) Foundation Trust and the University College of London Institute of Ophthalmology. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.

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Authors and Affiliations

  1. Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK

    Ethan S. Sen & Athimalaipet V. Ramanan

  2. School of Clinical Sciences, University of Bristol, Retinal Treatment and Research Unit, Bristol Eye Hospital, Lower Maudlin Street, Bristol, BS1 2LX, UK

    Andrew D. Dick

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  1. Ethan S. Sen
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E.S.S. researched data for and wrote the article. A.D.D. and A.V.R. contributed substantially to discussions of the article content and review or editing of the manuscript before submission.

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Correspondence to Athimalaipet V. Ramanan.

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Competing interests

A.D.D. declares that he has served as a consultant for Abbvie, Genentech and Novartis, has received grants from Novartis, and is a co-investigator for the SYCAMORE trial of adalimumab in patients with JIA-associated uveitis, which is funded by Health Technology Assessment and Arthritis Research UK. A.V.R. declares that he has received honoraria or speaker's fees from Abbvie, Novartis, Pfizer, Roche, and Swedish Orphan Biovitrum Pharmaceuticals, and that he is co-chief investigator of the SYCAMORE trial. E.S.S. declares no competing interests.

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Sen, E., Dick, A. & Ramanan, A. Uveitis associated with juvenile idiopathic arthritis. Nat Rev Rheumatol 11, 338–348 (2015). https://doi.org/10.1038/nrrheum.2015.20

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