What are the treatment guidelines for pediatric uveitis (inflammation of the uvea in children)?

From the Guidelines

Pediatric uveitis treatment should follow a stepwise approach, starting with topical corticosteroids as first-line therapy, and progressing to systemic therapy with methotrexate and biologic agents like adalimumab as needed, to rapidly control inflammation and prevent complications. The treatment of pediatric uveitis is crucial to prevent sight-threatening complications such as cataracts, glaucoma, and synechiae. According to the 2019 American College of Rheumatology/American Academy of Pediatrics guideline for the screening, monitoring, and treatment of juvenile idiopathic arthritis-associated uveitis 1, topical glucocorticoids are the initial treatment of choice.

Key Considerations

• For anterior uveitis, prednisolone acetate 1% is typically used, beginning with frequent dosing (every 1-2 hours) and gradually tapering based on clinical response.
• Cycloplegic agents like cyclopentolate 1% or atropine 0.5-1% are added to prevent synechiae formation and reduce pain.
• If topical therapy is insufficient after 2-3 months or if intermediate/posterior uveitis is present, systemic therapy is indicated, with methotrexate as the preferred first-line systemic agent, starting at 10-15 mg/m² weekly (maximum 25 mg), with folic acid supplementation 1.
• For inadequate response after 3 months, consider adding adalimumab (20 mg subcutaneously every 2 weeks for patients <30 kg; 40 mg for those ≥30 kg) 1.
• Systemic corticosteroids (prednisone 1-2 mg/kg/day, maximum 60 mg) may be used for severe inflammation or as a bridge therapy, but should be tapered within 3-4 weeks due to growth concerns.

Monitoring and Follow-up

Regular monitoring includes:

• Ophthalmologic exams every 1-3 months
• Laboratory tests for medication toxicity
• Assessment of growth and development This approach aims to rapidly control inflammation to prevent complications like cataracts, glaucoma, band keratopathy, and vision loss while minimizing treatment-related adverse effects in the developing child, as supported by the Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for juvenile idiopathic arthritis-associated and idiopathic chronic anterior uveitis 1.

From the FDA Drug Label

The safety and efficacy of HUMIRA were assessed in a randomized, double-masked, placebo-controlled study of 90 pediatric patients from 2 to < 18 years of age with active JIA-associated non-infectious uveitis (PUV-I). Patients received either placebo or 20 mg adalimumab (if < 30 kg) or 40 mg adalimumab (if ≥ 30 kg) every other week in combination with a dose of methotrexate Concomitant dosages of corticosteroids were permitted at study entry followed by a mandatory reduction in topical corticosteroids within 3 months. The primary endpoint was ‘time to treatment failure’. The criteria determining treatment failure were worsening or sustained non-improvement in ocular inflammation, or worsening of ocular co-morbidities HUMIRA significantly decreased the risk of treatment failure by 75% relative to placebo (HR = 0.25 [95% CI: 0.12,0.49])

The recommended treatment guideline for pediatric uveitis is 20 mg adalimumab (if < 30 kg) or 40 mg adalimumab (if ≥ 30 kg) every other week in combination with a dose of methotrexate, with a mandatory reduction in topical corticosteroids within 3 months 2.

• Key points:
  • Dosing: 20 mg or 40 mg every other week
  • Combination therapy: with methotrexate
  • Corticosteroid reduction: mandatory reduction in topical corticosteroids within 3 months
• Clinical response: HUMIRA significantly decreased the risk of treatment failure by 75% relative to placebo.

From the Research

Treatment Guidelines for Pediatric Uveitis

• The treatment of pediatric uveitis typically involves a step-ladder approach, with corticosteroids as the initial treatment option 3, 4, 5.
• Topical corticosteroids are often used as the first-line treatment, while systemic corticosteroids may be used in more severe cases of intermediate, posterior, or panuveitic uveitis 4, 5.
• In cases where corticosteroids are not effective or cannot be tapered, immunosuppressive therapy may be necessary to control the disease 3, 4.
• Methotrexate is often considered the first-line corticosteroid-sparing agent, and may be given as a subcutaneous weekly injection 3, 4.
• Biologic therapies, such as adalimumab and infliximab, may be used as second-line agents in children who are refractory to methotrexate or have severe disease at presentation 4.

Disease Management and Monitoring

• Pediatric uveitis can be asymptomatic and may become chronic, causing damage to ocular structures 6.
• Regular follow-up examinations are necessary to monitor disease activity and adjust treatment as needed 6.
• The Standardization of Uveitis Nomenclature (SUN) criteria may be used to classify and monitor uveitis in pediatric patients 6.
• Visual acuity and the number of cells in the anterior chamber can be used as measures of disease activity and visual function 6.

Complications and Morbidities

• Pediatric uveitis can lead to complications such as cataract, glaucoma, and amblyopia 6.
• Close monitoring and timely treatment are necessary to prevent or manage these complications 6.
• The impact of uveitis, its treatment, and its complications on the child and their family should be considered and addressed 6.