Paediatric non-infectious granulomatous uveitis: a retrospective cohort study

Abstract

Introduction: Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging.

Objective: The objective of this study was to evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis.

Methods: A retrospective chart review of non-infectious PGU occurring in children before the age of 16 years (recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023) was undertaken.

Results: We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with JIA, and 1 with Vogt-Koyanagi-Harada disease. The median age at diagnosis was 9.8 years (range 7.2-12.5). The M:F sex ratio was 0.52. The most common types of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%), particularly in cases of panuveitis. The most commonly used treatments were systemic CSs (72%) and MTX (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years.

Conclusion: We report the largest cohort of PGU. The cases of PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management.

Keywords: granulomatous; idiopathic; paediatric; sarcoidosis; uveitis.