What Is Juvenile Idiopathic Arthritis-Associated Uveitis?

Uveitis is the most common extra-articular (occurring outside of the joints) complication of juvenile idiopathic arthritis (JIA). Characterized by inflammation of the inner eye, uveitis can cause serious optical complications. If untreated, a child's vision could be affected. In extreme cases, uveitis may cause loss of vision or even blindness.

A focus on joint health in children with JIA is critical, but it's also important that you are aware of all of the ways arthritis can affect your child's body—especially their eyes.

While an immediate referral for an eye exam is a typical next step after a juvenile idiopathic arthritis diagnosis is made, and symptoms are not always noticeable at first, you should be aware of the possibilities and how JIA-associated uveitis (a.k.a. JIA-U, iridocyclitis) can progress over time.

Types of Uveitis

JIA-U can be a chronic or acute disease:

• Chronic anterior uveitis (CAU) is the most common form of JIA-associated uveitis. Roughly 10% to 20% of children with JIA develop this condition.
• Acute anterior uveitis (AAU) occurs in children with spondyloarthritis, including psoriatic arthritis. AAU comes and goes and will present suddenly with symptoms. It generally does not require systemic treatment.

Uveitis Symptoms

The condition can affect one or both eyes, but three out of four uveitis patients develop inflammation in both eyes.

While JIA patients can experience obvious joint pain and swelling, chronic uveitis eye symptoms may not appear early on or may be mild enough that no eye problems seem evident.

But as the condition progresses, children may have severe enough eye inflammation to develop the following symptoms:

• Sensitivity to bright light, both indoors and outdoors
• Aching or pain in the eyes
• Blurred vision
• More tear production than normal, which can lead to red or swollen eyes

Chronic uveitis related to JIA can also create an inflammatory environment in the eye that can scar tissues and cause significant eye diseases, such as:

• Glaucoma
• Cataracts
• Band keratopathy (calcification of the cornea)
• Macular edema (fluid in the retina)
• Blindness

Causes

JIA-associated uveitis is caused by JIA itself, with the autoimmune disorder producing systemic inflammation that progresses and damages the eyes.

In some cases, medications to treat uveitis can also cause vision complications. In particular, corticosteroids—while an effective front-line treatment—may contribute to the formation of cataracts.

Uveitis is more common in girls (up to 80% of cases), probably because JIA itself affects girls and boys at a roughly 3:2 ratio.

While the pathogenesis of JIA-U is not fully understood, some research has shown JIA patients having increased numbers of antibodies against the iris and retina, possibly explaining the autoimmune response attacking the eyes.

Diagnosis

Once a JIA diagnosis is initially confirmed, the child should immediately see a pediatric ophthalmologist for a thorough eye exam to check for inflammation. Early detection of JIA-U is critical.

Generally, an ophthalmologist will use a special microscope called a slit lamp to shine a thin beam of light into an eye to examine the interior. The presence of inflammation generally indicates JIA-U.

Onset

No signs of eye inflammation soon after receiving a JIA diagnosis does not mean that uveitis is not a possibility.

While uveitis can occur concurrently with JIA, that is not always the case. JIA-U can occur up to a year before JIA is diagnosed or even many years after diagnosis.

In about half of uveitis patients, the condition manifests within five months of JIA symptoms setting in. About three-quarters of uveitis diagnoses happen within one year of JIA symptoms setting in.

Most cases come from early-onset JIA, usually between ages 4 and 6.

Treatment

Initial treatment of children with JIA-associated uveitis typically includes topical corticosteroids (eye drops) to help reduce inflammation.

Medications commonly used for JIA can also be used for uveitis:

• Methotrexate is the usual first-line systemic immunosuppressive drug.
• Tumor necrosis factor inhibitor (TNFi) biologics may also be used, particularly the monoclonal antibodies infliximab and adalimumab.

Prognosis

If JIA-U is detected early and treated promptly, most children will have a good prognosis.

In one study of JIA-U patients treated with a variety of methotrexate, adalimumab, and topical corticosteroids, 82% of patients achieved inactive uveitis after one year, and 80% after two years. Almost 60% of patients achieved an inactivity period greater than six months.

Along with treatment, it is vital to schedule regular, ongoing eye examinations with a pediatric ophthalmologist. When determining an appropriate examination schedule, your healthcare provider will consider the type of arthritis, the age of the child at the onset of JIA, the duration of symptoms of JIA, any potential uveitis symptoms, and the amount of eye inflammation.

A Word From Verywell

While there's no guarantee that any child with juvenile idiopathic arthritis will develop uveitis, it's important for the healthcare provider diagnosing JIA to refer the child to a pediatric ophthalmologist for ongoing monitoring. Early detection and treatment of uveitis is worth stressing again, as it offers the child the best chance for normal vision throughout life.