BackgroundChildhood onset uveitis comprises a group of rare inflammatory disorders characterized by clinical heterogeneity, chronicity, and uncertainties around long term outcomes. Standardized, detailed datasets with harmonized clinical definitions and terminology are needed to enable the clinical research necessary to stratify disease phenotype and interrogate the putative determinants of health outcomes. We aimed to develop a core routine clinical collection dataset for clinicians managing children with uveitis, suitable for multicenter and national clinical and experimental research initiatives.MethodsDevelopment of the dataset was undertaken in three phases: phase 1, a rapid review of published datasets used in clinical research studies; phase 2, a scoping review of disease or drug registries, national cohort studies and core outcome sets; and phase 3, a survey of members of a multicenter clinical network of specialists. Phases 1 and 2 provided candidates for a long list of variables for the dataset. In Phase 3, members of the UK's national network of stakeholder clinicians who manage childhood uveitis (the Pediatric Ocular Inflammation Group) were invited to select from this long-list their essential items for the core clinical dataset, to identify any omissions, and to support or revise the clinical definitions. Variables which met a threshold of at least 95% agreement were selected for inclusion in the core clinical dataset.ResultsThe reviews identified 42 relevant studies, and 9 disease or drug registries. In total, 138 discrete items were identified as candidates for the long-list. Of the 41 specialists invited to take part in the survey, 31 responded (response rate 78%). The survey resulted in inclusion of 89 data items within the final core dataset: 81 items to be collected at the first visit, and 64 items at follow up visits.DiscussionWe report development of a novel consensus core clinical dataset for the routine collection of clinical data for children diagnosed with non-infectious uveitis. The development of the dataset will provide a standardized approach to data capture able to support observational clinical studies embedded within routine clinical care and electronic patient record capture. It will be validated through a national prospective cohort study, the Uveitis in childhood prospective national cohort study (UNICORNS).

The challenges of childhood uveitis lie in the varied spectrum of its clinical presentation, the often asymptomatic nature of disease, and the evolving nature of the phenotype alongside normal physiological development. These issues can lead to delayed diagnosis which can cause significant morbidity and severe visual impairment. The most common ocular complications include cataracts, band keratopathy, glaucoma, and macular oedema, and the various associated systemic disorders can also result in extra-ophthalmic morbidity. Pediatricians have an important role to play. Their awareness of the various presentations and etiologies of uveitis in children afford the opportunity of prompt diagnosis before complications arise. Juvenile Idiopathic Arthritis (JIA) is one of the most common associated disorders seen in childhood uveitis, but there is a need to recognize other causes. In this review, different causes of uveitis are explored, including infections, autoimmune and autoinflammatory disease. As treatment is often informed by etiology, pediatricians can ensure early ophthalmological referral for children with inflammatory disease at risk of uveitis and can support management decisions for children with uveitis and possible underling multi-system inflammatory disease, thus reducing the risk of the development of irreversible sequelae.

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