Granulomatous Features in Juvenile Idiopathic Arthritis-Associated Uveitis is Not a Rare Occurrence

Abstract

Background

Juvenile idiopathic arthritis (JIA)-associated uveitis is the most common form of intraocular inflammation with systemic involvement in childhood. Textbooks tend to describe it as non-granulomatous anterior uveitis. Here, we report the percentage of granulomatous features in JIA-related uveitis among patients at a uveitis referral center.

Methods

We conducted a retrospective study of all patients with JIA-related uveitis who were seen at the Center for Ophthalmic Specialized Care in Lausanne between 2000 and 2020. The sample comprised pediatric patients with bilateral anterior uveitis diagnosed as JIA-related with positive antinuclear antibody (ANA) titers. Exclusion criteria were a positive Mantoux or Quantiferon^® test or elevated serum lysozyme and/or angiotensin converting enzyme (ACE) levels. The presence of one of the following features qualified the uveitis as granulomatous: granulomatous keratic precipitates (KPs) of mutton-fat type, smaller granulomatous KPs, or Koeppe nodules. The amount of inflammation was measured using laser flare photometry.

Results

Thirty-eight patients were identified (mean age 13.34 ± 2.71 years; all female). In 12 patients (31.5%), the uveitis presented granulomatous features at presentation or during follow-up in at least one eye. Two of the 12 (16%) granulomatous patients had only light or no systemic involvement. The mean flare was 136.3±149 ph/ms, which is much higher than granulomatous uveitis entities of other origin reported in the literature.

Conclusion

JIA related uveitis can have a granulomatous presentation in about one third of patients with the Oligoarticular type and ANA positive, seen in a uveitis referral center. The presence of granulomatous features in bilateral uveitis in ANA-positive pediatric patients should not exclude the diagnosis of JIA-related uveitis, even in the absence of systemic involvement, as this entity presents a much more severe uveitis than other granulomatous anterior uveitis entities.

Keywords:

• JIA-related uveitis
• anterior uveitis
• non-granulomatous anterior uveitis
• granulomatous anterior uveitis
• laser flare photometry

Ethics

The study was performed in accordance with the ethical standards laid down in the Declaration of Helsinki and in accordance with the IRB of our institution (Ethical Committee of the Centre of Ophthalmic Specialised care and Montchoisi Teaching Centre, protocol number: EC-COS-MTC-20-12-01) authorizing retrospective, anonymous and non-interventional studies. Parental informed consent statement was signed,authorizing review of medical records.

Disclosure

The authors report no conflicts on interest in this work.