Eye Checks for children with JIA

Screening for Uveitis in children with JIA

Some children with chronic arthritis may also develop inflammation within the eye, a condition known as uveitis.  Adults who develop uveitis suffer a condition that is disruptive, as frequent visits to hospital eye clinics may be necessary, and often painful, but rarely leads to loss of vision. In adults uveitis is recognised quickly because the eye hurts or goes red or causes noticeable changes to the sight. Adult patients usually consult an ophthalmologist in a timely fashion and appropriate treatment is begun for irreversible damage has taken place.

For unknown reasons, the uveitis that occurs in children with JIA rarely hurts or makes the eye go red, and children are very slow to report symptoms of blurring. Children with uveitis therefore often suffer irreversible damage to the eye if there are delays in detecting the disease. Consequently, children with arthritis, who are at risk of uveitis, are screened for several years in order to detect disease before it causes irreversible damage to the eyes.

The development of new methods of controlling uveitis once it appears is very encouraging. No new treatments appear, unfortunately, to reduce the incidence of uveitis, so making sure children with JIA attend their uveitis screening checks remains as important as ever.

Contemporary risks of visual loss in JIA Uveitis

Before effective treatments of uveitis were invented in the 1950s, about 40% of children lost sight from the complications of chronic uveitis. This figure also suggests that the majority of children with uveitis did not suffer significant visual loss despite poorly controlled inflammation for some years and the lack of effective treatments.

Nowadays, it is still true that the majority of children have a benign course with mild inflammation lasting on average 5 years and who require no more treatment than topical steroids. About 40% still continue to have disease that is much more prolonged and either have complications, such as cataract, at the first visit, or develop complications after years of persistent inflammation. Recent studies show 20% of children with uveitis will get cataracts and this rate has not changed significantly over the last twenty years. Rates of visual loss are however improving and are less than 5%. It is now unlikely for a child who has normal vision at the onset of uveitis to develop blindness.

Complications of Uveitis

Mild uveitis in patients with JIA does not cause symptoms or damage with any great speed. The initial signs are so mild that they require a microscopic examination of the eye on a slit-lamp, and it may take several weeks for the inflammation to cause the mildest blurring of vision. The problem with JIA-uveitis is that the inflammation tends to persists for years rather than weeks, and complications, when they arise are often severe and irreversible. Treatment is primarily aimed to reduce long-term complications rather than restore function and reduce symptoms as in a painful immobilising arthritis.

The main dangers to the eye arising from chronic inflammation such as uveitis include the development of cataracts, changes in the pressure of the eye, which may rise too high [glaucoma] or fall too low [hypotony]. If inflammation spreads to the back of the eye, then the sight can be directly reduced by swelling of the nerves of the retina that serve vision [macular oedema].

The outcome of uveitis has improved over the last 50 years for several reasons. Firstly, patients are screened more efficiently and so less damage is present at the first visit at which uveitis is diagnosed. Secondly, there are a wider range of drugs available to control inflammation both within the joints in the eye. Usually the same drugs can be used to treat both arthritis and uveitis, but for reasons that are not well understood, some immunosuppressive drugs act differently on the inflammation in the eye and the joints. Thirdly, as doctors gain more experience of drugs that have been used for a long time, they tend to be more confident about starting treatment in milder cases and earlier on in the course of disease this may have contributed to a fall in the number of children who develop visual loss from uveitis. Fourthly there have been considerable changes in the surgical techniques used to remove cataracts, treat glaucoma and repair retinal detachments and this has helped the outcome of children requiring surgery for the complications of uveitis.

There is no single treatment for uveitis. There is a very wide range of severity of disease at the onset and disease can last for months or for more than twenty years with a wide range of final outcomes. No medical treatments can cure the disease, all they can do is control it while it is there. The ophthalmologist therefore needs to continually reassess with the patient and family the purpose of treatment, and how to balance the risks of treatment against the risks of under treating uveitis. These decisions are often not easy as the child with uveitis often has no complaints, appears to see normally, and the eye looks normal. Decisions about treatment often have to be made with uncertain knowledge as they are often made to prevent complications developing some years in the future. Children with persistent uveitis may still be losing sight in their twenties and more aggressive treatment early in the course of disease may have prevented these long-term complications.

When new drugs are invented that appear to control disease more effectively than older drugs there can be a clamour for their use to be more widespread. It can be some years before it is clear whether new drugs have any advantage over present treatments. No drugs are without side-effects and the main concerns when treating children with uveitis is whether there are significant risks on growth, and, if treatments need to be given for 10 years or more whether there are life-long risks that are unknown. We do not know the long-term side effects of newer drugs and it will take decades to do so and this has to be balanced against their effectiveness in small groups of patients. It is likely that as many children will benefit from the more efficient and timely use of drugs such as steroids and methotrexate as will benefit from the biologic agents that are presently available.

There will continue to be a variety of treatments of uveitis as new drugs are developed for both uveitis and arthritis, and as more long-term experience of contemporary drugs becomes available. The treatment options suitable for each child will continue to vary through the course of disease and families will always have to deal with difficult management decisions in the absence of much published scientific fact. The clinical experience of rare conditions is always spread thinly and it will always be important that patients, their families and the doctors involved share their experiences about how best to lessen the impact of these diseases.

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The eye and JIA written by: Mr Clive Edelsten, Consultant Ophthalmologist, Ipswich Hospital NHS Trust

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