BASICS
DESCRIPTION
• Juvenile idiopathic arthritis (JIA)-related uveitis is a group of idiopathic arthritides with an onset before 16 years of age that persists longer than 6 weeks.
• There are 7 subtypes of JIA
– Systemic arthritis: Arthritis in one or more joints accompanied or preceded by fever and accompanied by one of the following: Evanescent, erythematous rash, generalized lymph node enlargement, hepatomegaly and/or splenomegaly, or serositis
– Oligoarthritis: Arthritis in 1–4 joints in the first 6 months of disease. Oligoarthritis is further subcategorized as persistent oligoarthritis (affecting not more than 4 joints throughout the disease course) or extended oligoarthritis (affecting a total of more than 4 joints after the initial 6 months)
– Polyarthritis (RF negative): Arthritis in 5 or more joints in the initial 6 months and a negative test for rheumatoid factor (RF)
– Polyarthritis (RF positive): Arthritis in 5 or more joints in the initial 6 months and a positive test for RF on 2 occasions at least 3 months apart during the first 6 months of disease
– Psoriatic arthritis (PsA): Arthritis and psoriasis or arthritis and at least 2 of the following: Dactylitis, nail-pitting or onycholysis, or psoriasis in a first-degree relative
– Enthesitis-related arthritis (ERA): Arthritis and/or enthesitis (tenderness at the insertion of a tendon, ligament, fascia, or capsule to bone) with at least 2 of the following: Presence or history of sacroiliac joint tenderness, HLA-B27 antigen, onset of arthritis in male over 6 years of age, acute (symptomatic) anterior uveitis, or history of ankylosing spondylitis, ERA, sacroiliitis with inflammatory bowel disease, Reiter syndrome or acute anterior uveitis in a first-degree relative
– Undifferentiated arthritis: Arthritis that fulfills criteria in no category or in 2 or more of the above categories
EPIDEMIOLOGY
Incidence
The exact incidence of JIA is unknown. The reported incidence varies from 0.008–0.226 per 1,000 children per year.
Prevalence
• The exact prevalence of JIA is unknown. The reported prevalence varies from 0.07–4.01 per 1,000 children.
• The prevalence of uveitis within known JIA populations varies from 4–38% worldwide. Analysis of pulled data suggests a cumulative prevalence of uveitis of 8.3% of JIA patients.
RISK FACTORS
Risk factors for developing JIA uveitis include ANA positivity, early age at onset of arthritis (<6 years), and female sex.
Genetics
• HLA-DRB1 has been linked with an increased susceptibility to develop JIA.
• HLA-DRB1∗13 has been associated with the development of JIA uveitis.
PATHOPHYSIOLOGY
• Abnormal immune responses have been documented in JIA patients, but the precise etiology remains unknown.
• Serologic abnormalities such as antineutrophil antibody (ANA), RF, and anticyclic citrullinated peptide (anti-CCP) have implicated self-targeting antibodies, but the exact target remains unknown.
ETIOLOGY
Etiology is unknown.
COMMONLY ASSOCIATED CONDITIONS
See 7 subtypes in “Description”.
DIAGNOSIS
HISTORY
• Many patients with JIA uveitis do not have any ocular symptoms, which is the reason for regular ophthalmic screening examinations according to disease subtype.
• One should inquire about decreased vision, history of pink eye, and photophobia.
PHYSICAL EXAM
• Patients may be categorized according to type of uveitis as standardized by the SUN group (see “Additional Reading”).
– Acute anterior uveitis: Anterior uveitis that is sudden in onset and has <3 months duration
– Recurrent anterior uveitis: Anterior uveitis of less than 3 months duration but with recurrent episodes that occur >3 months apart without medication
– Chronic anterior uveitis: Anterior uveitis that lasts longer than 3 months
– Anterior uveitis with vitreitis: Anterior uveitis with vitreitis and this does not include snowbanking or spillover cells in the vitreous
• Ophthalmic features of JIA uveitis may include:
– Bilateral anterior uveitis with a chronic course; in most cases, both eyes are involved within a few months of each other.
– It is usually a nongranulomatous uveitis; however, recent studies suggest that granulomatous inflammation may be more common than previously thought, particularly in African–Americans and ANA-positive children.
– One should look for uveitic complications, such as posterior synechiae, cataract, band keratopathy, glaucoma, hypotony, and cystoid macular edema (CME).
– Chronic anterior uveitis is the most common, followed by acute anterior uveitis, recurrent anterior uveitis, and finally, anterior uveitis with vitreitis.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• There are no confirmatory tests since JIA is a diagnosis of exclusion. One may consider the following tests if the diagnosis has not already been made:
• Erythrocyte sedimentation rate, C-reactive protein, platelet count, ANA, RF, and anti-CCP. One may consider laboratory tests for Lyme disease and sarcoidosis as well.
Imaging
One may consider optical coherence tomography if CME is suspected.
DIFFERENTIAL DIAGNOSIS
• Sarcoidosis is the disease that most closely mimics JIA uveitis.
• Other diseases to consider include: Lyme disease, trauma, Kawasaki disease, and herpetic keratouveitis.
TREATMENT
MEDICATION
First Line
• Topical corticosteroids are first line therapy to treat anterior uveitis (treat cells, not flare). The dose is titrated according to the cellular reaction, the presence of posterior synechiae, and band keratopathy. The goal is to use the least amount of steroid that will maintain quiescence. If the steroid drop must be used more than 4 times per day, then a second line drug should be used.
• Short-acting mydriatics and cycloplegics should be used. Short acting allows the pupil to move and not develop synechiae either in a dilated or non-dilated state. Be careful not to induce amblyopia by constant cycloplegia.
Second Line
• Periocular injections of steroids may be necessary. But they carry a higher risk of glaucoma and cataract formation.
• Oral corticosteroids should not be used for prolonged periods of time given the adverse impact on growth and bony development.
• Naproxen may have some benefit as a steroid-sparing agent; it is the most frequent nonsteroidal anti-inflammatory drug prescribed for patients with JIA.
• Methotrexate (MTX) is the most commonly used agent when adjunctive therapy is needed. It usually takes 4–8 weeks for an effect to be seen. Liver enzymes and CBC should be monitored every 4–6 weeks.
• Cyclosporine and azathioprine have also been used with success.
• The biologics are also being increasingly utilized. Although etanercept works well for joint disease, in a randomized, controlled trial, it was no better than placebo for JIA uveitis. However, infliximab and adalimumab appear to be effective for treating JIA uveitis.
ADDITIONAL TREATMENT
General Measures
If uveitic complications develop, surgery may be warranted to address these. See “Surgery/Other Procedures.”
SURGERY/OTHER PROCEDURES
• Cataract formation is the most common vision-threatening complication of JIA uveitis. Up to 60% of children with JIA uveitis develop cataract due to chronic inflammation and/or corticosteroid use.
– In the past, pars plana lensectomy, anterior vitrectomy and removal of the capsule were performed. This required lifelong dependence on aphakic spectacles or contact lenses.
– Recently, success has been achieved with phacoemulsification, posterior capsulorrhexis, anterior vitrectomy, and intraocular lens placement (often accompanied by an injection of triamcinolone acetonide into the anterior chamber).
• Glaucoma may be managed by topical therapy; but if drops do not control the intraocular pressure, then trabeculectomy and antimetabolite use or glaucoma drainage device placement may be necessitated.
• Band keratopathy may require chelation therapy, but recurrences are common.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Pediatric ophthalmologist
• Pediatric rheumatologist
• Consider uveitis specialist
Patient Monitoring
Screening for eye disease once JIA is diagnosed is mandatory. Referral to ophthalmologist should be performed promptly. Current guidelines for screening are summarized in Table.
PATIENT EDUCATION
Parents should be taught that the ocular inflammation in JIA is often asymptomatic and therefore, regular screening appointments are necessary. They should also be taught that any pink eye should not be dismissed as viral until evaluated by an ophthalmologist.
PROGNOSIS
In some of the more recent studies, over 90% of eyes with JIA uveitis, at final visit, had a visual acuity of 20/40 or better; and over 97% of patients (using better-seeing eye) had 20/40 or better vision.
COMPLICATIONS
See “Surgery/Other Procedures”
ADDITIONAL READING
• Jabs DA, Nussenblatt RB, Rosenbaum JT; Standardization of Uveitis Nomenclature (SUN) Working Group. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol. 2005;140:509–516.
• Kesen MR, Setlur V, Goldstein DA. Juvenile idiopathic arthritis-related uveitis. Int Ophthalmol Clin. 2008; 48(3):21–38.
• Sabri K. Saurenmann RK, Silverman ED, et al. Course, complications, and outcome of juvenile arthritis-related uveitis. J AAPOS. 2008; 12:539–545.
• American Academy of Pediatrics Section on Rheumatology and Section on Ophthalmology: Guidelines for ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics. 1993;92(2):295–296.
Table *.* Frequency of Ophthalmologic Visits for Children With Juvenile Rheumatoid Arthritis (JRA) and Without Known Iridocyclitis∗
CODES
ICD9
• 364.00 Acute and subacute iridocyclitis, unspecified
• 364.3 Unspecified iridocyclitis
• 714.30 Chronic or unspecified polyarticular juvenile rheumatoid arthritis
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