The Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) initiative made recommendations for the diagnosis and treatment of juvenile idiopathic arthritis (JIA)-associated uveitis. The recommendations have been published in the Annals of the Rheumatic Diseases.
The SHARE initiative established a committee of 9 experienced pediatric rheumatologists and 3 ophthalmology experts to formulate the recommendations. The committee accepted 22 recommendations with >80% agreement among experts, including 3 for diagnosis, 5 for disease activity measurements, 12 for treatment, and 2 for future recommendations.
Beginning in February 2015, SHARE researchers searched electronic databases by keywords; relevant studies were allocated to members of the expert group and assessed for methodological quality. Of the 1323 papers yielded from the literature search, 117 were selected to support recommendation development. Final recommendations were accepted at an agreement threshold of ≥ 80% among experts, including 3 ophthalmologists.
Diagnosis and Screening of JIA-Associated Uveitis
- Experts recommended that any patient with suspected JIA be screened for uveitis according to a contemporary and audited protocol and advocated the use of formal screening procedures at any centers that see patients with JIA.
- The frequency of ophthalmological follow-up should be dictated by disease severity and decided in conjunction with an expert ophthalmologist.
- All patients with JIA should receive screening from an ophthalmologist at least every 3 months for at least 1 year following cessation of immunosuppressant treatment, given the potential risk for new onset uveitis or uveitis recurrence.
Disease Activity Measurement in JIA-Associated Uveitis
- Researchers recommended proper communication between ophthalmologists and pediatric rheumatologists to effectively monitor disease activity and treatment outcomes.
- Panelists also emphasized the need for shared outcome measures to help guide systemic treatment decisions.
- Because there is no widely accepted definition of inactive disease for JIA-related uveitis, experts recommend that the primary treatment goal should be no cells in the anterior chamber. Patients presenting with macular or disk edema, ocular hypotony, or rubeosis iridis may require anti-inflammatory treatment even in the absence of anterior chamber cells.
- Experts suggested that clinicians wait to reduce systemic immunosuppression until patients achieve 2 years of inactive disease without topical steroids.
Treatment of JIA-Associated Uveitis
- Active uveitis in JIA usually requires immediate treatment.
- Topical corticosteroids should be used as first-line treatment in anterior uveitis.
- Systemic immunosuppression is recommended if (1) poor prognostic factors are present at the first visit or persist later in the disease course or (2) inactivity could not be reached within 3 months or inflammation reactivated during steroid dose reduction.
- Methotrexate (MTX) was recognized as the first-choice medication for systemic immunosuppression.
- Experts recommended adding or switching to biological treatment in the case of MTX inefficacy or intolerance.
- In patients with DMARD-resistant or refractory uveitis, experts suggested the use of anti-tumor necrosis factor (TNF) therapy.
- Switching between different anti-TNF treatments may be helpful in patients refractory to the first anti-TNF treatment.
- In cases of lack of efficacy for anti-TNF strategies, experts suggested testing patients for antidrug antibodies and drug trough level; patients with no antibodies but low trough levels may benefit from an increased dose or shortened intervals.
- Experts recommended tocilizumab, rituximab, and abatacept as potential options in patients unresponsive to previous anti-TNF therapy.
- Etanercept should not be used for JIA-associated uveitis.
- Experts also noted that topical and systemic nonsteroidal anti-inflammatories have no demonstrable effect as monotherapy but may be used as additional therapy.
The panel also put forward 2 recommendations for future directions in JIA-related uveitis, stating that (1) validated outcome measures for JIA-associated uveitis should be developed and (2) JIA-associated uveitis requires future research in the form of controlled clinical trials. Researchers emphasized the low level of quantitative evidence for several of the recommendations; 13 of 22 were based either on descriptive study or expert opinion alone, rather than meta-analyses of cohort or case-control studies. Therefore, future directions in longitudinal studies and clinical trials will be significant in validating diagnostic and management strategies in patients with JIA-associated uveitis.
Constantin T, Foeldvari I, Anton J, et al. Consensus-based recommendations for the
management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative [published online March 28, 2018]. Ann Rheum Dis. doi:10.1136/annrheumdis-2018-213131