Anterior uveitis

Juvenile idiopathic arthritis

CLINICAL PRESENTATION AND DIAGNOSIS

Juvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatoid arthritis (JRA), is the leading cause of uveitis in children (198). The average age at diagnosis is 6 years, with the eye involvement usually following the onset of joint disease. Up to 20% of JIA patients develop uveitis but the incidence varies by subgroup. Pauciarticular disease (fewer than five joints), female sex, and circulating antinuclear antibodies all increase the risk of developing uveitis. Children with JIA often have no symptoms other than joint pain. Some children may complain of photophobia or chronic red eyes, but often the disease is symptom free in its early stages. The uveitis is usually bilateral and chronic, with infrequent acute attacks. Since the uveitis associated with JIA is often symptom free, routine ophthalmologic screening is imperative.1

Diagnosis of JIA is usually made by a rheumatologist, and patients are then sent to an ophthalmologist for uveitis evaluation.

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198 White blood cells floating in the anterior chamber in a patient with JIA.(Courtesy of Peter Buch,CRA.)

MANAGEMENT/TREATMENT

The first line of treatment is topical corticosteroids to reduce the inflammation, and mydriatic agents to help keep the pupil mobile and prevent scarring to either the cornea or lens. Topical corticosteroids are used frequently during exacerbations and tapered as the inflammation subsides. If topical steroids are insufficient to control the inflammation, depot steroid injections or even systemic steroids are used. In cases of uveitis where significant doses of steroids are needed to control the inflammation, and they cannot be easily tapered, immunosuppressive agents are often used as an adjunct therapy.

PROGNOSIS

Complications of JIA-associated uveitis are very common. More than 50% of patients develop band keratopathy (deposits of calcium in the cornea related to chronic inflammation). These are usually not visually significant unless they encroach on the visual axis (central corneal), and they can be treated with chelation if necessary. Cataracts can develop in up to one-third of patients. These are particularly troublesome because replacement of the cataract with an intraocular lens remains debatable due to the concern for ongoing inflammation in these eyes. Until recently a diagnosis of JIA has been a contraindication to lens implantation; however, some ophthalmologists are attempting lens implantation with newer (less inflammatory) lenses. Glaucoma occurs in about 20% of these patients and does not respond well to medical therapy. Early detection and treatment of JIAassociated uveitis are of utmost importance to preserve vision in these children.2

Juvenile spondyloarthropathies

CLINICAL PRESENTATION AND DIAGNOSIS

This category of disease accounts for approximately 15% of pediatric anterior uveitis cases. It includes juvenile ankylosing spondylitis, juvenile Reiter’s syndrome, juvenile psoriatic arthritis, ulcerative colitis, and Crohn’s disease. Boys are more commonly affected, usually after 8 years of age. These patients often present with acute recurrent uveitis that is bilateral and asymmetric. Some juvenile ankylosing spondylitis patients can present with a severe acute anterior uveitis and hypopyon formation.

A large majority of these patients are positive for the HLA-B27 haplotype and most are RF negative.

MANAGEMENT/TREATMENT AND PROGNOSIS

Treatment of the uveitis is similar to JIA and includes topical corticosteroids and mydriatic agents. The acute recurring bouts of inflammation tend to taper off as these children age. Also, the first episode of uveitis is often the most severe, and if treated early, the visual prognosis is very good.

Sarcoidosis

CLINICAL PRESENTATION AND DIAGNOSIS

There are two distinct subgroups of sarcoidosis that present with ocular inflammation. Childhood sarcoidosis is seen in children less than 5 years old, is more common in girls, and is characterized by arthritis with a skin rash. There is a striking predominance of Caucasian children affected by this early-onset sarcoidosis. In contrast, adult-type sarcoidosis is also seen in children, most commonly between the ages of 8 and 15 years, and is three times more common in African–American children than in Caucasians. This form is seen with equal frequency in boys and girls. These children often present with pulmonary findings, and can have hepatosplenomegaly and lymphadenopathy. Both types frequently have ocular involvement (199), but uveitis in these patients is often asymptomatic. It is important to note that, while anterior uveitis is the most common presentation, sarcoidosis can present with intermediate, posterior, or pan (multiple layer) uveitis.3

Laboratory investigations include angio- tensin-converting enzyme (ACE) levels, serum lysozyme levels, chest X-ray, and gallium scanning of the lungs and lacrimal glands. ACE levels must be age-matched because children have higher ACE levels than adults. Definitive diagnosis is made by biopsy of a nodule showing noncaseating granulomas.

MANAGEMENT/ TREATMENT AND PROGNOSIS

Treatment of the anterior uveitis consists of topical corticosteroids and mydriatic agents. Often systemic corticosteroids that are used to treat pulmonary complications can control the ocular disease. While dramatic improvement is seen clinically with systemic and topical steroids, the chronic nature of the uveitis associated with sarcoidosis often leads to the development of both cataracts and glaucoma. Both of these complications can be treated surgically with good results.

Herpetic iridocyclitis

CLINICAL PRESENTATION AND DIAGNOSIS

Both herpes simplex virus (HSV) and herpes zoster virus (HZV) can cause anterior uveitis. The inflammation is generally monocular, can be either acute or chronic, and is often associated with an increase in IOP. Herpetic uveitis typically occurs in conjunction with significant corneal involvement. Children typically present with photophobia and decreased vision.4,5 HSV and HZV uveitis can also present with concomitant hyphema (200).

Diagnosis is usually confirmed by corneal culture, and treatment is directed at the corneal disease to prevent scarring.

MANAGEMENT/ TREATMENT AND PROGNOSIS

Treatment generally consists of topical or systemic antiviral agents, corticosteroids, and mydriatics. Corticosteroids should be administered later in the course of the disease as they can prevent corneal epithelial wound healing.

Prognosis is good, with the ultimate visual outcome depending on the level of corneal scarring. Glaucoma can occur with active inflammation and is usually well treated with medical management. In cases of HZV uveitis, sector iris atrophy can be seen (201).

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199 Multiple iris nodules in sarcoidosis. (Courtesy of Peter Buch,CRA.)

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200 Gonioscopic photograph of a layered hyphema in a patient with HZV iritis.(Courtesy of Peter Buch,CRA.)

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