Anterior uveitis occurs in association with a variety of systemic conditions. Since the clinical features present on ophthalmic examination may not point toward a specific cause, the emphasis on general medical history and physical examination, the use of directed laboratory testing, and a referral to a pediatric rheumatologist, are appropriate. [1, 2]
In this article, anterior uveitis associated with juvenile idiopathic arthritis (JIA) and entities related to human leukocyte antigen B27 (HLA-B27) are covered, including juvenile ankylosing spondylitis (AS), reactive arthritis (also referred to as Reiter syndrome), and inflammatory bowel disease. Also included are sarcoidosis, which may present with a nongranulomatous anterior uveitis, Blau syndrome, acute tubulointerstitial nephritis and uveitis, and Kawasaki disease. Brief coverage is given to uveitis related to systemic viral infections.
The pathophysiology of these entities is diverse, and the specific entities are covered in more detail below. For example, poorly controlled uveitis related to JIA may progress to band keratopathy, hypotony, and phthisis, whereas the iritis related to Kawasaki disease is self-limited and generally benign.
Causes of anterior uveitis in children may be classified broadly as infectious and noninfectious. The common and important infectious causes are due to viruses (eg, herpes simplex, herpes zoster) and following systemic viral syndromes (eg, mumps). Uveitis associated with herpes simplex often is accompanied by keratitis, making the diagnosis straightforward. Uveitis in children with herpes zoster usually occurs in the setting of immune suppression, such as AIDS. [3, 4, 5]
Noninfectious causes of childhood anterior uveitis are more common; the strongest association is with JIA. In JIA, the patient may be asymptomatic as the process quietly proceeds to band keratopathy, cataract, and glaucoma. Spondyloarthropathies (eg, AS, psoriatic arthritis, reactive arthritis, inflammatory bowel disease) also are associated strongly with nongranulomatous anterior uveitis. Sarcoidosis is an important cause of panuveitis but may present as an isolated nongranulomatous anterior uveitis.
Approximately 6% of all cases of uveitis occur in children, with most cases occurring in association with JIA and the spondyloarthropathies. Uveitis can occur in 4 of the 7 categories of JIA, including oligoarthritis, rheumatoid factor (RF)-negative polyarthritis, enthesitis-related arthritis, and psoriatic arthritis.
Oligoarthritis affects 4 or fewer joints and typically occurs in young girls. Oligoarthritis is the most common type of JIA in Europe and North America. Uveitis most often accompanies this form of the disease and is seen in about 10-30% of patients. Antinuclear antibodies are common in this group of children. Uveitis occurs in up to 10% of children with RF-negative polyarthritis JIA. Enthesitis-related arthritis affects the attachments of ligaments and tendons to the bone. This form of JIA typically affects older boys. Uveitis is often unilateral with a sudden onset; children are often symptomatic. Approximately 10% of children with psoriatic arthritis develop uveitis. In these children, the uveitis is usually chronic and asymptomatic.
Of patients with AS, 20-30% develop uveitis; of patients with reactive arthritis, 12-37% develop uveitis; and, of patients with inflammatory bowel disease, 2-9% develop uveitis.
Uveitis associated with sarcoidosis is significantly less common in children compared to JIA-related causes and uveitis related to spondyloarthropathy. In one large series of childhood uveitis, it accounted for less than 1% of cases.
Uveitis in children and adolescents is less common than in adults. However, the reported percentage of children affected varies widely, ranging from 2.2-33.1% of all patients with uveitis. In a large study from Israel, anterior uveitis accounted for 13.4% of all cases of uveitis affecting children and adolescents.
Morbidity in childhood anterior uveitis may result from lack of treatment or from overzealous treatment.
The primary causes of ocular and visual morbidity in pediatric anterior uveitis are similar among the various entities, with the primary differences related to severity, chronicity, treatment success, complications, and age of onset.
Acute episodes of inflammation may be self-limited and benign or may cause anterior and posterior synechiae, with secondary glaucoma. Chronic anterior uveitis additionally may cause band keratopathy, cataract, spillover anterior vitreitis, and cystoid macular edema. Severe glaucoma and phthisis are the most feared complications.
Sarcoidosis affects African Americans about 10 times more often than it affects whites. However, most young children with uveitis are white.
AS is 2-3 times more common in boys than in girls.
Reactive arthritis is at least 5 times more common in boys than in girls.
Sarcoidosis is slightly more common in girls than in boys.
Oligoarthritis JIA is more common in females than in boys, with a girl-to-boy ratio of 3-4:1.
Mean age of onset of AS is about 10 years and 10-11 years in psoriatic arthritis.
Children (and parents/guardians) with childhood anterior uveitis require prolonged education regarding the signs and symptoms of disease activity. It is important to stress the need for lifestyle changes to decrease and prevent exacerbations and to preserve current remaining vision and the need for appropriate pain management. Children complain less regarding visual symptoms and require regular eye visits to check for signs of ocular inflammation.
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