047. Idiopathic uveitis – A paediatric cohort

Abstract

Background: Uveitis involves inflammation of the structures of the uveal tract, which can lead to visual disability. Juvenile idiopathic arthritis (JIA) related uveitis is the commonest cause in children. In most UK centres, paediatric rheumatology and ophthalmology services work together to investigate non-JIA uveitis and provide immunomodulatory therapy for refractory cases. All cases of non-JIA uveitis need exclusion of underlying causes, including systemic autoimmune disease and infection. Even though no underlying cause is usually found, the aetiology is thought to be autoimmune. A greater understanding of the disease processes may help to better target appropriate therapies. Aims: To determine demographics and disease characteristics in a cohort with refractory non-JIA uveitis. Methods: A retrospective case note analysis of patients with refractory non-JIA uveitis under a tertiary paediatric rheumatology service since 2012. Refractory disease was defined as those requiring methotrexate or biologic therapy. Results: We identified 20 patients with refractory non-JIA uveitis. The median age at data analysis was 15.5y (3-21y); 65% female; 65% White British ethnic origin, 5% each of Indian, Pakistani, Black African and Other Black origins. 85% had bilateral uveitis. 70% had anterior uveitis, 15% pan uveitis, 10% intermediate and 5% posterior. Antinuclear antibodies(ANA) were recorded in 90%. Of these 50% were ANA positive at some point but only 17% strongly positive (1:160). Extractable nuclear antigen antibodies(ENA) and double-stranded DNA, measured in 45% and 70% respectively, were negative in all. Serum ACE was measured in 80% and was above normal range in 44%. The highest values were associated with a diagnosis of systemic sarcoidosis but not ocular sarcoidosis (diagnosed based on ocular findings). Only 1 patient received ocular biopsy. 95% had exclusion of tuberculosis using Quantiferon gold testing. A variety of infectious organisms were screened for with no significant positive results. 75% of cases were diagnosed as idiopathic, 15% related to sarcoid (2 systemic, 1 ocular), 5% to psoriasis and 5% probably toxocara related (based on ocular findings). Conclusions: The largest group with idiopathic uveitis were White British and female, likely a reflection of female preponderance in most autoimmune conditions. The commonest presentation was bilateral anterior uveitis. There were no clear immunological blood markers associated with disease although 50% were ANA positive; some in this group may have been JIA presenting as uveitis where systemic immunosuppression may have suppressed development of joint symptoms. The commonest disease association was sarcoidosis. No infective causes were found. Serum screening, however, may not yield positive results, especially in ocular toxocara. Ocular biopsy may be warranted in refractory cases to look for evidence of infective causes not otherwise identified, in order to target appropriate therapy.