Uveitis, Anterior, Childhood 

  • Author: R Christopher Walton, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: May 2, 2012
 

Background

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 subspecialist, such as a 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.

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Pathophysiology

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.

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Epidemiology

Frequency

United States

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.

International

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.

Mortality/Morbidity

  • 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.

Race

Sarcoidosis affects African Americans about 10 times more often than it affects whites. However, most young children with uveitis are white.

Sex

  • AS is 2-3 times more common in males than in females.
  • Reactive arthritis is at least 5 times more common in males than in females.
  • Sarcoidosis is slightly more common in females than in males.
  • Oligoarthritis JIA is more common in females than in males, with a female-to-male ratio of 3-4:1.

Age

  • Most children with oligoarthritis JIA are younger than 4 years, while children with RF-negative polyarthritis JIA are somewhat older. Enthesitis-related arthritis typically affects older boys.[6, 7]
  • Mean age of onset of AS is about 10 years and 10-11 years in psoriatic arthritis.
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Contributor Information and Disclosures
Author

R Christopher Walton, MD  Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Peter H Spiegel, MD, to the development and writing of this article.

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