eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Juvenile Idiopathic Arthritis

Author: Manolette R Roque, MD, MBA, DPBO, FPAO, President and CEO, Chief of Service, Ocular Immunology and Uveitis, Consulting Staff, Cornea and Refractive Surgery, Eye Republic Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines Co; Consulting Staff, CME Liaison, Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center
Coauthor(s): Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic; Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School; C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
Contributor Information and Disclosures

Updated: Oct 24, 2008

Introduction

Background

Approximately 6% of all cases of uveitis arise in children1 The most frequent cause of chronic intraocular inflammation among children is juvenile idiopathic arthritis (JIA)-associated uveitis2 A unifying classification, juvenile idiopathic arthritis (JIA), encompassing juvenile chronic arthritis and juvenile rheumatoid arthritis (JRA), has been developed by consensus.

Chronic iridocyclitis occurs in 10-20% of all patients with JIA.3,4,5,6 Chronic uveitis characteristically is asymptomatic in children with JIA, leading to insidious but progressive morbidity and possible blindness. The involved eyes often are white and quiet appearing, yet 30-40% of patients with JIA-associated uveitis experience severe loss of vision as a consequence of their condition.

JIA, as defined by the American Rheumatism Association (ARA), is the presence of arthritis (chronic, seronegative, and peripheral) before age 16 years, of at least 3 months duration, when other causes have been excluded. It is classified by 1 of 3 types of onset.7

Oligoarticular (pauciarticular) onset JIA (40-60%) is common in girls (5:1). Peak age of onset is at age 2 years. Four or fewer joints are involved during the first 6 months of the disease (often asymmetric). Oligoarticular onset commonly involves the knees and, less frequently, the ankles and wrists. The arthritis may be evanescent, rarely destructive, and radiologically insignificant. Approximately 75% of these patients test positive for antinuclear antibody (ANA). This mode of onset rarely is associated with systemic signs. A high risk for uveitis exists.8

Polyarticular onset JIA (20-40%) is common in girls (3:1). Peak age of onset is at age 3 years. It involves 5 or more joints during the first 6 months of the disease. Polyarticular onset JIA commonly involves the small joints of the hand and, less frequently, the larger joints of the knee, ankle, or wrist. Asymmetric arthritis may be acute or chronic and may be destructive in 15% of patients. Immunoglobulin M (IgM) rheumatoid factor (RF) is present in 10% of children with this JIA subgroup. It is associated with subcutaneous nodules, erosions, and a poor prognosis. Approximately 40% of these patients test positive for ANA. Systemic symptoms, including anorexia, anemia, and growth retardation, are moderate. An intermediate risk for uveitis exists.

Systemic onset JIA (10-20%) is equal frequency in boys and girls and can appear at any age. Symmetric polyarthritis is present and may be destructive in 25% of patients. Hands, wrists, feet, ankles, elbows, knees, hips, shoulders, cervical spine, and jaw may be involved. ANA is positive in only 10% of the patients. Systemic onset is associated with fever (high in evening and normal in morning), macular rash, leukocytosis, lymphadenopathy, and hepatomegaly. Pericarditis, pleuritis, splenomegaly, and abdominal pain less commonly are observed. A low risk for uveitis exists. 

See related CME at Updates on Pediatric Rheumatoid Arthritis. 

Pathophysiology

The cause of uveitis and arthritis in JIA remains unknown.9 Akin to many other autoimmune diseases, the target antigen is unidentified. Immune reactions to ocular antigens (S antigen or iris antigen) have been studied; however, their actual role (active or passive) is unknown. The course of the disease may be short and limited or progressive and severe.

Frequency

United States

JIA has an estimated prevalence of about 113 cases per 100,000 children. It is estimated that JIA afflicts 60,000-70,000 children, but only a minority develop eye disease. Incidence of eye disease in the JIA population is uncertain, but it is believed to be around 10%.7

Mortality/Morbidity

Morbidity in JIA-associated uveitis may result either from lack of treatment or from overzealous treatment. Mortality may result from the latter.

Race

No known racial predilection exists.

Sex

A strong predilection exists for girls. The girl-to-boy ratio is 4:1.

Age

JIA is a childhood disease.

  • By definition, JIA occurs in children younger than age 16 years.
  • In view of the fact that the ocular disease can follow the systemic disease by numerous years, a lot of patients are well beyond their teens when they are examined and treated for uveitis.10

Clinical

History

Always complete a thorough history of new patients. The ocular immunology and uveitis survey form, developed by Dr. C Stephen Foster, provides a complete checklist (present illness, past medical history, family history, and review of systems) and may assist the physician in the history. The patient questionnaire may be downloaded from the Massachusetts Eye and Ear Infirmary Immunology and Uveitis Service.

  • Chief complaint and history of present illness
    • Many patients are referred first by a pediatrician or rheumatologist and often are asymptomatic. Typically, patients have no pain or photophobia and the eye appears white.
    • Asymptomatic patients may recall previous insignificant symptoms that may be useful in determining the duration of the ocular disease. Therefore, ask specific questions regarding past ocular history, such as previous episodes of pink eye or conjunctivitis, blurry vision, ocular pain, or abnormal pupil size/shape.
  • Past medical history
    • Crucial to establish chronicity and to determine the subtype of JIA
    • Age at onset
    • Specific joints involved and number of joints
    • Other systemic manifestations are as follows:
      • Fever, rash, lymphadenopathy, fatigue, weight loss, hepatosplenomegaly, pericardial effusion, pleural effusion
      • Anemia
      • Growth retardation, delay of secondary sexual characteristics
      • Chronic low back pain, diarrhea, psoriasis
    • Current systemic medications for JIA
  • Past ocular history
    • Previous episodes and treatment
    • Previous surgeries
    • Previous complications
      • Cataract
      • Glaucoma
      • Band keratopathy
      • Cystoid macular edema (CME)
  • Review of systems
    • General - Weight loss, fatigue, fever
    • Skin - Rash, nodules, changes in nails
    • Neck - Lymphadenopathy
    • Respiratory - Cough, wheezing
    • Cardiac - Chest pain/discomfort, dyspnea
    • Gastrointestinal - Hepatomegaly, diarrhea, frequent bowel movements
    • Genitourinary - Delayed secondary sexual characteristics
    • Musculoskeletal - Muscle or joint pain, arthritis, back pain, limitation of motion

Physical

  • Ocular manifestations - Perform a complete ophthalmic examination.
    • Vision - Best-corrected visual acuity, including near acuity
    • Anterior uveitis
      • Cells and flare; chronic flare (very common)
      • Nongranulomatous uveitis (>90%)
      • Bilateral (70-80%)
      • Iridocyclitis in approximately 90% of patients; rarely panuveitis or vitritis
      • Chronic smoldering or recurrent disease in greater than 90%; rarely acute monophasic course (<5%)
    • Conjunctiva and sclera - Most patients have no conjunctival injection even during acute exacerbations.
    • Cornea
      • Band keratopathy - Corneal degeneration that derives its name from the distinctive appearance of calcium deposition in a band across the central cornea
      • Keratic precipitates - Small-medium, rarely mutton fat
    • Iris - Posterior synechiae; pupillary membrane; rarely may develop Koeppe nodules
    • Pupil examination
    • Lens - Posterior subcapsular cataracts
    • Posterior uveitis
      • Dilated fundus examination - Vitritis, CME, hypotony maculopathy
      • Anterior vitreous (anterior vitreous cells not uncommon)
    • Others - Intraocular pressure, secondary glaucoma (open angle or pupillary block), hypotony
  • Articular manifestations
    • Assessment must consist of a quick evaluation of the skin and joints (warmth, redness, effusion, and deformity). Identify the particular joint and number involved.
    • The ophthalmologist evaluating the patient may perform this; however, the pediatrician or rheumatologist is expected to execute a complete musculoskeletal examination.

Causes

The cause of uveitis and arthritis in JIA remains unknown. Akin to many other autoimmune diseases, the target antigen is unidentified. Associated factors may include the possibility of infectious triggers, a genetic predisposition, an autoimmune response, psychological stress, female sex, and hormone interaction.11,9

More on Uveitis, Juvenile Idiopathic Arthritis

Overview: Uveitis, Juvenile Idiopathic Arthritis
Differential Diagnoses & Workup: Uveitis, Juvenile Idiopathic Arthritis
Treatment & Medication: Uveitis, Juvenile Idiopathic Arthritis
Follow-up: Uveitis, Juvenile Idiopathic Arthritis
Multimedia: Uveitis, Juvenile Idiopathic Arthritis
References
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References

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Further Reading

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Keywords

uveitis, juvenile idiopathic arthritis, juvenile idiopathic arthritis associated uveitis, JIA-associated uveitis, JIA uveitis, JRA-associated uveitis, JRA uveitis, juvenile rheumatoid arthritis, juvenile rheumatoid arthritis associated uveitis, vision loss, blindness, chronic iridocyclitis, chronic intraocular inflammation

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Contributor Information and Disclosures

Author

Manolette R Roque, MD, MBA, DPBO, FPAO, President and CEO, Chief of Service, Ocular Immunology and Uveitis, Consulting Staff, Cornea and Refractive Surgery, Eye Republic Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines Co; Consulting Staff, CME Liaison, Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center
Manolette R Roque, MD, MBA, DPBO, FPAO is a member of the following medical societies: American Academy of Ophthalmic Executives, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, American Society of Ophthalmic Administrators, American Uveitis Society, International Ocular Inflammation Society, Philippine Medical Association, Philippine Ocular Inflammation Society, and Philippine Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic
Disclosure: Nothing to disclose.

Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School
Disclosure: Nothing to disclose.

C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
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.

CME Editor

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.

 
 
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