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Juvenile rheumatoid arthritis and glaucoma
Last reviewed: 05.07.2025
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Juvenile rheumatoid arthritis is a common cause of uveitis in children, which often leads to increased intraocular pressure and glaucoma.
Depending on the number of affected joints and the presence of systemic manifestations during the first 3 months from the onset of the disease, there are 3 subtypes of juvenile rheumatoid arthritis with different risks of uveitis development. Juvenile rheumatoid arthritis with systemic manifestation, or Still's disease, is an acute systemic disease manifested by skin rash, fever, polyarthritis, hepatosplenomegaly, leukocytosis and polyserositis; it is usually found in boys under 4 years of age. Girls more often develop oligo-, pauciarticular (less than 5 joints are affected) and polyarticular (5 or more joints are affected) forms of juvenile rheumatoid arthritis, in which there are no systemic manifestations.
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Epidemiology of juvenile rheumatoid arthritis
The incidence of uveitis in juvenile rheumatoid arthritis ranges from 2% to 21%. Uveitis is usually not present in Still's disease, or systemic juvenile rheumatoid arthritis. Anterior uveitis is more common in patients with the pauciarticular form (19% to 29%) than in those with the polyarticular form (2% to 5%) of juvenile rheumatoid arthritis. Children with pauciarticular or monoarticular forms of joint manifestations account for more than 90% of patients with juvenile rheumatoid arthritis-related uveitis. Secondary glaucoma develops in approximately 14% to 22% of patients with chronic anterior uveitis associated with juvenile rheumatoid arthritis.
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What causes juvenile rheumatoid arthritis?
Increased intraocular pressure and development of glaucoma in patients with juvenile rheumatoid arthritis most often occur as a result of synechial closure of the anterior chamber angle. Open-angle glaucoma may develop with chronic inflammation of the trabecular meshwork, and steroid-induced glaucoma may develop with prolonged local treatment with glucocorticoids.
Symptoms of Juvenile Rheumatoid Arthritis
In 90% of patients with juvenile rheumatoid arthritis, uveitis develops after the arthritis. Because anterior uveitis in juvenile rheumatoid arthritis is mild, asymptomatic, and rarely causes redness of the eye, the disease may not be detected for a long time until decreased visual acuity, cataracts, or pupil deformity are noticed. In almost all cases, uveitis in juvenile rheumatoid arthritis is bilateral.
Course of the disease
Juvenile rheumatoid arthritis-associated uveitis is a chronic disease that is difficult to treat. In patients with juvenile rheumatoid arthritis, there is no direct relationship between the activity of the eye lesion and the joint involvement. The longer the disease duration, the higher the risk of developing secondary complications, such as band keratopathy, cataracts, and glaucoma. The previously poor prognosis in children with inflammatory glaucoma has improved somewhat due to the development of more effective surgical techniques.
Ophthalmological examination
Band keratopathy is found in almost 50% of children with anterior uveitis, which is probably associated with the chronic course of the disease. Anterior uveitis in patients with juvenile rheumatoid arthritis is nongranulomatous in the vast majority of cases. However, sebaceous precipitates on the cornea and Koeppe nodules are found in rare cases. Precipitates are usually located in the lower half of the cornea. Patients often have signs that can lead to the development of glaucoma: miosis due to the presence of posterior synechiae or pupillary membranes, iris bombage, and peripheral anterior synechiae. Anterior and posterior subcapsular cataracts develop in about 1/3 of patients. When examining the posterior segment of the eye in patients with juvenile rheumatoid arthritis, papillitis and cystoid macular edema can be detected, which can lead to a decrease in visual acuity.
Diagnosis of juvenile rheumatoid arthritis
Differential diagnosis of chronic anterior uveitis in children is carried out with sarcoidosis, pars planitis, HLA B27-associated diseases and idiopathic anterior uveitis.
Laboratory research
Almost 80% of patients with anterior uveitis in juvenile rheumatoid arthritis have antinuclear antibodies and no rheumatoid factor.
Treatment of juvenile rheumatoid arthritis
Primary treatment of intraocular inflammation in patients with juvenile rheumatoid arthritis includes topical glucocorticoids and cycloplegics to prevent adhesions. Periocular or systemic glucocorticoids are often needed to treat anterior uveitis. Nonsteroidal anti-inflammatory drugs (NSAIDs) are also used orally. Methotrexate is used alone or in combination with other immunosuppressants (prednisolone or cyclosporine) to treat ocular or joint manifestations of juvenile rheumatoid arthritis. Newer biologics, etanercept (Enbrel) and infliximab (Remicade), have been shown to be effective against joint damage in juvenile rheumatoid arthritis. Studies of their effectiveness in uveitis are currently underway.
When intraocular pressure increases in juvenile rheumatoid arthritis, treatment with antiglaucoma drugs is performed. The effectiveness of drug therapy in patients suffering from juvenile rheumatoid arthritis is initially 50%, but over a long period of time, drug control is achieved in only 30% of patients. Laser iridotomy or surgical iridectomy may be required to eliminate pupillary block in the presence of posterior synechiae. If drug therapy is ineffective, surgical treatment is necessary. To improve the results of surgical intervention, the operation should be performed with control of intraocular inflammation for at least 3 months. Children with juvenile rheumatoid arthritis undergo trabeculectomy and implantation of tubular drainage. Better results of trabeculectomy have been noted with the use of antimetabolites. Trabeculodialysis in a small group of patients with juvenile rheumatoid arthritis was effective in reducing intraocular pressure for up to 2 years.