The goals of treatment of uveitis in children are to eliminate the inflammation of the eye before ocular complications occur and to monitor for ocular and systemic adverse effects of the treatment. It is important to note that although the presence of cells in the anterior chamber indicates active inflammation, flare (protein) may persist long after the inflammation has been successfully treated.
Infectious diseases and malignancies producing uveitis should be identified and treated appropriately. Treatment of noninfectious uveitis is discussed in the following sections.
Management of Inflammation
Anterior segment inflammation is initially treated with topical corticosteroid and mydriatic/cycloplegic agents. Because topical corticosteroids do not penetrate well into the vitreous or posterior segment, sub-Tenon injections of a corticosteroid may be useful in the treatment of intermediate or posterior uveitis. Short courses of oral corticosteroids may be used, but long-term use is usually avoided because of the potential for significant ocular and systemic adverse effects. Corticosteroid intravitreal implants containing either fluocinolone acetonide or dexamethasone have been used successfully to treat posterior uveitis in children.
Table 24-6 Laboratory Tests and Imaging for Various Types of Uveitis
Glaucoma and cataract formation are the most serious ocular complications of any corticosteroid therapy. In general, more potent topical corticosteroids are more likely to increase intraocular pressure. Periocular injections of corticosteroids can elevate intraocular pressure for weeks to months after injection. Cataracts and glaucoma are reported adverse effects of intravitreal steroid implants. Risks of long-term systemic corticosteroid use in children include growth retardation, osteoporosis and bone fractures, cushingoid appearance, diabetes mellitus, peptic ulcers, myopathy, hypertension, altered mental status, idiopathic intracranial hypertension, and increased risk of infection. Patients may also require increased doses of corticosteroids during times of stress to avoid an addisonian crisis.
Systemic immunosuppressive therapy is beneficial in treating both uveitis and arthritis. It can sometimes reduce or eliminate the need for steroids. The therapy should be undertaken in cooperation with a pediatric specialist familiar with the use of immunosuppressive and immunomodulatory medications. In patients with JIA, immunosuppressive drugs reduce the risk of vision loss from uveitis.
Methotrexate is the most common antimetabolite used to treat arthritis and uveitis in children. Less commonly used antimetabolites include azathioprine and mycophenolate mofetil. These agents inhibit nucleic acid synthesis by a variety of mechanisms. Gastrointestinal disturbance is the most common adverse effect of oral methotrexate and can be alleviated by switching to subcutaneous injections. Oral folic acid supplementation is often recommended for patients using methotrexate. Hepatic toxicity, interstitial pneumonitis, and cytopenia are rare but serious adverse effects of methotrexate use.
Biologic drugs for the treatment of uveitis are used with increasing frequency to suppress the immune system in children. The 2 classes of biologic medications are tumor necrosis factor α (TNF-α) inhibitors and cell-specific antibodies. The TNF-α inhibitors most commonly used to treat uveitis are infliximab and adalimumab, which are monoclonal IgG antibodies against TNF-α. Commonly used cell-specific antibodies include abatacept (antibody to CD80 and CD86) and rituximab (antibody to interleukin-2). Etanercept should be avoided because new-onset inflammatory eye disease (uveitis, scleritis, optic neuritis) has been associated with its use, caused by poor drug efficacy and/or adverse effect of the drug. In general, TNF-α inhibitors are used before cell-specific antibodies. TNF-α inhibitors should not be used during periods of active infection. All these biologic drugs used to treat uveitis require intravenous infusion, except for adalimumab, which can be given subcutaneously. There is concern that children and adolescents treated with TNF-α blockers may be at increased risk for malignancies; however, a recent study showed that children with JIA are at increased risk for malignancies unrelated to the use of biologic drugs.
Beukelman T, Haynes K, Curtis JR, et al; Safety Assessment of Biological Therapeutics Collaboration. Rates of malignancy associated with juvenile idiopathic arthritis and its treatment. Arthritis Rheum. 2012;64(4):1263–1271.
Gregory AC II, Kempen JH, Daniel E, et al; Systemic Immunosuppressive Therapy for Eye Diseases Cohort Study Research Group. Risk factors for loss of visual acuity among patients with uveitis associated with juvenile idiopathic arthritis: the Systemic Immunosuppressive Therapy for Eye Diseases Study. Ophthalmology. 2013;120(1):186–192.
Klotsche J, Niewerth M, Haas JP. Long-term safety of etanercept and adalimumab compared to methotrexate in patients with juvenile idiopathic arthritis (JIA). Ann Rheum Dis. 2016;75(5):855–861.
Reiff A, Kadayifcilar S, Özen S. Rheumatic inflammatory eye diseases of childhood. Rheum Dis Clin North Am. 2013;39(4):801–832.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.