Key Points
- Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA) and can be a chronic or acute disease.
- Chronic anterior uveitis (CAU) develops in 10–20% of children with JIA, is usually asymptomatic, and there is rarely external evidence of inflammation.
- Acute anterior uveitis (AAU) is a distinctly different form of uveitis. It is typically associated with HLA–B27 and occurs in children with spondyloarthritis (i.e., those with enthesitis-related or psoriatic arthritis).
- AAU differs from CAU in that AAU is episodic, unilateral, characterized by the sudden onset of erythema, pain, and photophobia, and generally does not require systemic treatment.
- Uncontrolled CAU can lead to sight-threatening complications such as synechiae, cataracts, and glaucoma in 25–50% and vision loss in 10–20% of children with uveitis.
- Early detection through regular ophthalmic screening with timely and appropriate treatment can improve visual outcomes and prevent ocular complications.
Diagnosis
Grade Definitions on Strength of Recommendation and Guide to Interpretation
| Strength | For the patient | For the clinician | |
|---|---|---|---|
| Strong | “should use” or “should be used” | Most individuals in this situation would want the recommended course and only a small proportion would not. | Most individuals should receive the recommended course of action. Formal decision aids are not likely to be needed to help individuals make decisions consistent with their values and preferences. |
| Conditional | “may be used” or “may consider” or “Y (less preferred drug) may be used instead of X (preferred drug)” or “may consider Y instead of X (preferred drug)” | The majority of individuals in this situation would want the suggested course, but many would not. | Different choices would be appropriate for different patients. Decision aids may be useful in helping individuals in making decisions consistent with their values and preferences. Clinicians should expect to spend more time with patients when working towards a decision. |
Grade Definitions of Quality and Certainty of the Evidence
| Quality Grade | Definition |
|---|---|
| High | Very confident that the true effect lies close to that of the estimate of the effect. |
| Moderate | Moderately confident in the effect estimate. The true effect is likely to be close to the estimate of effect, but there is a possibility that it is substantially different. |
| Low | Confidence in the estimate is limited. The true effect may be substantially different from the estimate of effect. |
| Very low | Very little confidence in the effect estimate. The true effect is likely to be substantially different from the estimate of effect. |
Table 1. Terms, definitions, and medication interventions
| Terms/medications | Definition/name |
|---|---|
| Terms | |
| Controlled uveitis | Inactive OR grade <1+ anterior chamber cells without new complications due to active inflammation per Standardization of Uveitis Nomenclature criteriaa |
| Complications due to active inflammationb | New development of peripheral anterior synechiae, posterior synechiae, inflammatory membranes, or cystoid macular edema |
| Additional signs of active inflammation | Fresh keratic precipitates, increased flare, and hypotony |
| Complications representing cumulative damage | Cataract, glaucoma/elevated intraocular pressure, hypotony, or sequelae of keratic precipitates (hyalinized spots or ghost keratic precipitates)c |
| Loss of controlb | Increase of anterior chamber cells to grade 1+ or more or new signs of inflammation/complications of inflammationa |
| New uveitis activity | No history of prior uveitis or loss of control of previously controlled uveitis |
| Systemic therapy | Nonbiologic DMARDs and biologics |
| Medications included in the guideline | |
| Glucocorticoids | Topical eye drops Systemic (all oral) |
| Nonbiologic DMARDs | Methotrexate Leflunomide Mycophenolate Cyclosporine |
| Biologic DMARDs | Monoclonal TNF inhibitor (adalimumab, infliximab) Etanercept Abatacept Tocilizumab |
b Loss of control of uveitis, active uveitis, and complications can lead to partial or permanent vision loss.
c These are not reversible changes and should not be indications to change treatment in the absence of active inflammation.
Figure 1. A, Ophthalmic screening examinations; B, Ophthalmic monitoring examinations
Figure 2. Recommendations for topical glucocorticoids in patients with juvenile idiopathic arthritis (JIA) and chronic anterior uveitis (CAU)
