Juvenile Idiopathic Arthritis Uveitis
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Approximately 6% of all cases of uveitis arise in children. [1] The most frequent cause of chronic intraocular inflammation among children is juvenile idiopathic arthritis (JIA)-associated uveitis. [2] 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.
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.
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]
Morbidity in JIA-associated uveitis may result either from lack of treatment or from overzealous treatment. Mortality may result from the latter.
No known racial predilection exists.
A strong predilection exists for girls. The girl-to-boy ratio is 4:1.
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]
Most vision-threatening morbidities in JIA are secondary to intraocular inflammation (eg, severity, chronicity). The development of JIA-associated uveitis heralds a poor prognosis.
Of affected eyes, 30-40% maintain long-term acuity greater than 20/40. Conversely, approximately 30-40% develop severe visual disability with acuity less than 20/200.
Poor prognosticators (higher risk for chronic iridocyclitis) include the following [11, 12] :
Female sex
Early onset pauciarticular arthritis–JIA, as follows:
Young age at disease onset
Pauciarticular arthritis
ANA – Positive
Others, as follows:
Posterior synechiae
Secondary glaucoma
Patients (and relatives) with JIA-associated uveitis require a significant amount of education regarding the signs and symptoms of disease exacerbation. Emphasize the need for lifestyle changes to prevent exacerbations, to preserve vision, and for appropriate pain control.
The need to maintain regular eye visits to check for ocular activity cannot be overemphasized, if one is to preserve the patients remaining useful vision.
For excellent patient education resources, see eMedicineHealth’s patient education articles Juvenile Rheumatoid Arthritis and Rheumatoid Arthritis Medications.
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Manolette R Roque, MD, MBA, FPAO Section Chief, Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke’s Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic; Director, AMC Eye Center, Alabang Medical Center
Manolette R Roque, MD, MBA, 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, Philippine Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.
Barbara L Roque, MD, DPBO, FPAO Senior Partner, Roque Eye Clinic; Chief of Service, Pediatric Ophthalmology and Strabismus Section, Department of Ophthalmology, Asian Hospital and Medical Center; Active Consultant Staff, International Eye Institute, St Luke’s Medical Center Global City
Barbara L Roque, MD, DPBO, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, Philippine Academy of Ophthalmology, Philippine Society of Cataract and Refractive Surgery, Philippine Society of Pediatric Ophthalmology and Strabismus
Disclosure: Nothing to disclose.
Elisabetta Miserocchi, MD
Disclosure: Nothing to disclose.
C Stephen Foster, MD, FACS, FACR, FAAO, FARVO 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, FARVO 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, Sigma Xi
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA)<br/>Received research grant from: Alcon; Aldeyra Therapeutics; Allakos Pharmaceuticals; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen.
Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department 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, Association for Research in Vision and Ophthalmology, American Glaucoma Society
Disclosure: Nothing to disclose.
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, Association for Research in Vision and Ophthalmology, Retina Society, American College of Healthcare Executives, American Uveitis Society
Disclosure: Nothing to disclose.
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, Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.
Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center
Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society
Disclosure: Nothing to disclose.
The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.
Juvenile Idiopathic Arthritis Uveitis
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