Manifestations of JIA: JIA-Associated Uveitis, sJIA & Lung Involvement in sJIA

PRSYM—At the virtual 2021 Pediatric Rheumatology Symposium (PRSYM), three speakers discussed JIA-associated uveitis, systemic JIA (sJIA) and pulmonary involvement in sJIA.

Eyes

Mindy Lo, MD, PhD, assistant professor of pediatrics at Harvard Medical School and director of the pediatric rheumatology fellowship training program at Boston Children’s Hospital, began the session by discussing guidelines for screening for JIA-associated uveitis.

The ACR guideline published in 2019 notes that patients who were identified as high risk for uveitis include those with anti-nuclear antibodies (ANAs), who had JIA onset prior to age 7 and a disease duration of less than or equal to four years.¹ European guidelines from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE) indicate that, although several screening protocols have been published, no evidence exists that any one protocol is superior to another.² Thus, the exact method for screening for uveitis may not be of utmost importance. What is essential is thinking to screen for this condition in a systematic manner.

Dr. Lo said disease activity in patients with JIA can be a relevant marker of potential ophthalmologic involvement. In a study of 98 patients with JIA-associated uveitis followed over the course of six years, researchers found a 73% concordance between active uveitis and arthritis. This finding indicates that arthritis activity in patients with JIA should prompt evaluation of uveitis activity.³

Regarding treatment, Dr. Lo stated a three-month benchmark is important in several respects. First, if steroid treatment alone does not result in uveitis inactivity within three months or if there is reactivation of inflammation with steroid tapering during this interval, systemic immunosuppression, such as with a tumor necrosis factor (TNF) α inhibitor, is warranted. Second, in cases in which cataract surgery is indicated, it’s important to achieve at least three months of uveitis inactivity prior to surgery.

With respect to treatment options, a number of mostly retrospective studies indicate that adalimumab may lead to higher rates of remission and lower rates of relapse of JIA-associated uveitis than infliximab. With regard to treatments beyond TNF inhibitors, options may include tocilizumab and abatacept. Janus kinase inhibitors, such as baricitinib, are currently under investigation as possible treatments for JIA-associated uveitis.

When to stop treatment is a question many rheumatologists face when caring for patients with JIA-associated uveitis. In a study of 98 patients with JIA-associated uveitis, factors associated with achieving uveitis inactivity included JIA onset at older than 4 years, uveitis onset at older than 5 years and adalimumab use. Although no specific laboratory parameters were predictive of achieving uveitis inactivity, decreasing arthritis activity was associated with uveitis inactivity.⁴

Systemic Disease

In the next portion of the session, Michael Ombrello, MD, tenure-track investigator, Translational Genetics and Genomics Unit, Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Bethesda, Md., discussed systemic JIA (sJIA). This condition manifests with a chronic inflammatory arthritis and fever, plus at least one of the following: salmon pink rash, generalized lymphadenopathy, organomegaly or serositis.