Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood, and of JIA extraarticular manifestations, JIA-associated uveitis (JIA-U) is the most common.1 Uncontrolled JIA-U can lead to blindness. Therefore, emphasis is placed on screening and treatment of JIA-U. This article will review the fundamentals of JIA and JIA-U, the screening guidelines for JIA-U, and the recommended consensus treatment algorithm.
Juvenile idiopathic arthritis subtypes and uveitis risk
There are several subtypes of JIA, including systemic, polyarthritis, oligoarthritic, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis. The three most common types of these subtypes are systemic JIA, oligoarticular JIA, and polyarticular JIA. Of patients with oligoarticular JIA, up to 10-20% will develop uveitis, and those that are ANA positive are at higher risk of developing uveitis.
Uveitis in JIA can present as either acute or chronic anterior uveitis. Acute anterior uveitis is associated with patients that are HLA-B27+ and is unilateral, episodic, and painful. Conversely, chronic anterior uveitis is often asymptomatic, painless, and vision-threatening, leading to complications such as synechiae, cataracts, band keratopathy, and glaucoma in 25-50% of patients and vision loss in 10-20%.2
Risk factors for vision-threatening uveitis include:1
- Male gender
- Young age of onset
- Short duration between onset of arthritis and uveitis
- Presence of synechiae at initial diagnosis
Screening and monitoring guidelines
The often asymptomatic nature of chronic anterior uveitis associated with JIA make it important to routinely evaluate and screen patients with JIA for signs of uveitis. Two hallmark guidelines together offer cohesive guidance for uveitis in patients with JIA. The first is the 2006 guidelines by the British Society for Paediatric and Adolescent Rheumatology (BSPAR) used by the Royal College of Ophthalmologists in the United Kingdom, and the second is the 2019 guidelines proposed by the American College of Rheumatology (ACR) and the Arthritis Foundation.2,3 The 2006 BSPAR guidelines advise initial screening in JIA suspect or recently diagnosed JIA patients, whereas the 2019 ACR guidelines describe further screening and monitoring in diagnosed JIA or JIA-U patients.
Figures 1 and 2 represent screening and monitoring workflow from the 2019 ACR Guidelines.2