Eye Complications of Juvenile Arthritis and Related Conditions

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In juvenile arthritis and related conditions, the eyes may be involved even when there is no evidence of active joint disease. Ocular complications may take several forms. Children with pauciarticular-onset, polyarticular-onset, and psoriatic arthritis are all at risk of developing eye inflammation (chronic anterior uveitis). In this condition, inflamed cells accumulate in the eye and the resultant irritation may cause damage to the colored part of the eye (iris), the lens, and other structures (see Fig. 5-1 in Chapter 5). The most worrisome aspect of this inflammation is that often it does not produce pain or redness, and so it may cause serious eye damage before it is detected.

The key to preventing serious eye damage in children with JA is careful screening for the presence of inflammatory cells by an ophthalmologist (a medical eye doctor). This is done with a special instrument called a slit lamp, which is not regularly available in the office of physicians who do not specialize in eye disease. An additional quick and easy test recommended by some ophthalmologists is for parents to shine a flashlight in the child's eyes at bedtime one night each week. If you shine a light in a normal child's eye, you will see the pupil (black center) shrink dramatically. It should shrink in a perfect circle. If one eye does not shrink or the circle is irregular, this might be evidence of eye involvement. Whatever the cause, any child whose pupils do not shrink equally when you shine a light in them should be evaluated by an ophthalmologist. This test may detect the onset of inflammation in the eye occurring between screening tests, but it is not a substitute for the screening tests.

Frequency of Routine Screening Ophthalmologic Examinations for Children with Juvenile Arthritis


ANA Status

Age at Onset

Length of Time with Disease

Screening Interval

Pauciarticular-onset and polyarticular-onset disease


Under age 7

Less than 4 years

Every 3 months

Systemic-onset JA


Over age 7 Under age 7 Over age 7

More than 4 years More than 7 years Less than 4 years More than 4 years Less than 4 years More than 4 years

Every 6 months Yearly

Every 6 months Yearly

Every 6 months Yearly Yearly Yearly

Note: These are recommendations for routine screening of children who are not known to have uveitis.

If the child has uveitis, the frequency of follow-up should be determined by the ophthalmologist.

Note: These are recommendations for routine screening of children who are not known to have uveitis.

If the child has uveitis, the frequency of follow-up should be determined by the ophthalmologist.

Physicians do not understand why some children get eye disease and others do not. There are certain genetic factors that seem to increase the risk. However, many children with these factors do not get eye disease, and some children without these factors do get eye disease. Attempts to understand why having ANA increases the risk have failed.

If you look carefully at the joint of a child with arthritis, the inflammation is centered in the synovium. This is the lining tissue, the normal function of which is to keep the joint clean of debris. Examination of the eyes of children with uveitis has shown that the inflammation is centered in the ciliary body. This is a tissue in the eye that serves to keep the fluid in the eye clean of debris. Since the two tissues serve a similar function, it is easy to understand how the same disease process might involve both. But if it were that simple, we would expect all children with arthritis to develop eye involvement. In fact, less than half ever have any evidence of eye inflammation. Clearly other factors play a role.

If uveitis is present, it should be treated aggressively. The normal first-line therapy is steroid eye drops. Often a short course of this treatment is enough to bring the disease under control. Unfortunately, steroid eye drops can damage the eye, possibly causing cataracts or glaucoma, if used for an extended period. Because of these complications, if it appears that a short course of treatment with steroid eye drops is inadequate, many ophthalmologists will recommend switching children with severe eye disease to immunosuppressive medications taken by mouth. Often methotrexate is the first medication used after corticosteroids have proven inadequate. Adalimumab and infliximab are also used in this situation with good efficacy. It is my personal preference to use adalimumab, as it seems to have a faster onset of action and produce a better and more sustained response. Some children respond best to the use of the combination of methotrexate and adalimumab. Since the adalimumab works faster, I usually add the methotrexate only when it is apparent that adalimumab alone is not enough. It is uncommon for children to require stronger medications; if they do, they should be under the care of experienced specialists in large centers.

Many children with JA are found to have mild uveitis, but if they are treated appropriately they do very well. However, if the uveitis is very severe, very resistant to therapy, or not found until very late, there is a risk of permanent blindness. Uveitis may occur in one or both eyes and may develop at a time where there is no evidence of active arthritis. This is why routine monitoring is so important—to find eye disease that you do not suspect is present, and to allow the doctor to begin therapy before too much damage accumulates. Eye doctor appointments should not be put off because you have not noticed any problem.

While eye involvement is common and some children develop serious eye involvement, since the introduction of more potent medications I have seen complete blindness in children with arthritis in only two situations: when children fail to go for screening examinations as recommended and when parents take their children off medication against the physician's advice because they are concerned the child might suffer side effects.

Eye involvement may also occur in children with spondyloarthropathies. However, these children usually get acute and painful eye disease (see Chapter 7). Because it is painful, it is usually rapidly detected. Frequent monitoring of children older than ten with spondyloarthropathies who do not have symptoms in their eyes is not required. In contrast, young children with psoriatic arthritis are at high risk of inflammatory eye disease and must be monitored just as if they had typical JA.

Eye disease may also be a complication of other rheumatic diseases in childhood, including sarcoidosis, systemic lupus erythematosus, and Sjogren's syndrome. Children with each of these conditions should have their eyes checked by an ophthalmologist routinely. In some cases, they will have symptoms of their eye disease; in other cases the eye disease may occur without the parents or child being aware of it. Some children develop uveitis without evidence of a rheumatic disease. Since this eye disease looks the same as the eye disease in children with JA, it is treated the same way. The list of diseases that may cause uveitis in childhood is quite long; however, the first step in evaluating a child who is found to have uveitis is a careful evaluation to make sure he or she does not have a rheumatic disease.

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