UNICORNS: Uveitis in childhood prospective national cohort study protocol [version 1; peer review: 1 approved, 1 approved with reservations]

Background: Childhood uveitis is a rare inflammatory eye disease which is typically chronic, relapsing-remitting in nature, with an uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long term outcome is lacking, including the predictors of therapeutic response or how to define disease control. Aims: To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes. Methods: UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected systematically. Participants and their families will also complete patient-reported outcome measures annually from recruitment. The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement. Discussion: Through this population based, prospective longitudinal study of childhood uveitis, we will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies will be enabled of outcome for affected children and families

Etiologies of Chronic Anterior Uveitis at a Tertiary Referral Center over 35 Years

PURPOSE: To describe the epidemiology of chronic anterior uveitis (CAU) at a tertiary center over 35 years. METHODS: Data regarding etiology of CAU was collected from medical records of patients evaluated between 1973-2007. Relative frequencies of each diagnosis of CAU were calculated. Linear regression analyses were performed on the common types of CAU. RESULTS: 5970 patients were evaluated between 1973-2007; 31% carried a diagnosis of CAU. Idiopathic disease was diagnosed in 54% of patients (39 to 72% annually), ocular sarcoidosis in 14% of patients (2 to 20% annually), Fuchs heterochromic iridocyclitis (FHI) in 12% of patients (4 to 22% annually), and juvenile idiopathic arthritis (JIA) in 6% of patients (2 to 13% annually). The frequency of diagnosis of idiopathic CAU decreased over time, with no significant change for sarcoidosis, FHI or JIA. An increase in frequency of diagnosis was observed for HLA-B27-related disease and uveitis related to multiple sclerosis and inflammatory bowel disease. CONCLUSIONS: The relative frequency of idiopathic disease has decreased over the past 35 years at our center. This may be related to an increase in the diagnosis of CAU associated with HLA-B27 positivity, inflammatory bowel disease (including family history) and multiple sclerosis. Despite the advances over the last 35 years, idiopathic disease still comprises at least 39% of our patients with CAU each year