Ophthalmologic Examinations in Children With JRA

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Guidelines for Ophthalmologic Examinations in Children With JRA

AMERICAN ACADEMY OF PEDIATRICS

http://www.aap.org/policy/04483.html

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Section on Rheumatology and Section on Ophthalmology

Chronic, nongranulomatous iridocyclitis is an important complication of

juvenile rheumatoid arthritis (JRA). [1-3] First reported by Ohm in

1910, the association between iridocyclitis and JRA has become well

established. The intraocular inflammation referred to as iridocyclitis

primarily affects the iris and ciliary body. Overall, the reported

incidence of iridocyclitis varies from 2% to 21% in children with JRA.

[4] The morbidity of iridocyclitis includes cataracts, glaucoma, band

keratopathy, and loss of vision. [5,6] Diagnosis of early iridocyclitis

is usually not possible by routine direct ophthalmoscopy. Slitlamp

examination detects the signs of active anterior chamber inflammation.

Guidelines for the schedule of routine serial slitlamp examination are

suggested for early detection of iridocyclitis.

The presentation of eye involvement in JRA may be asymptomatic or of an

insidious onset. The outcome has improved in the past 20 years. The

majority of children have a relatively good visual prognosis if the

iridocyclitis is detected and treated early. [6]

RISK FACTORS FOR IRIDOCYCLITIS

Articular Features

The diagnosis of JRA describes a heterogeneous group of arthritic

conditions with onset of disease before age 16 years. There are three

major subtypes of JRA: systemic onset, polyarticular onset, and

pauciarticular onset, defined by the clinical manifestations in the

first 6 weeks of the disease. [7] Fewer than 2% of children with

systemic-onset JRA have iridocyclitis. [4,5] Children with polyarticular

disease are at moderate risk, with 7% to 37% incidence of iridocyclitis.

The majority of children with iridocyclitis have pauciarticular disease.

[1-3,5] The onset of the iridocyclitis may precede the onset of the

arthritis in approximately 6% of cases. Rarely, it occurs in the absence

of arthritis after long-term follow-up. Iridocyclitis may be detected at

the time of initial diagnosis of arthritis; however, it most often

presents over the next 5 to 7 years. The highest risk of iridocyclitis

is most commonly within 2 years of the onset of arthritis. [5] Children

with JRA remain at risk for iridocyclitis into adulthood. There are

reports of iridocyclitis diagnosed more than 20 years after the onset of

the arthritis. [5] Iridocyclitis does not usually parallel the activity

of joint disease. [8]

Age, Gender

The majority of children at risk for developing iridocyclitis are young

females with pauciarticular-onset JRA. [1-3,5] The peak age of onset of

the arthritis in this group is age 2 to 5 years, with subsequent

development of iridocyclitis within the next 5 to 7 years. [5]

Immunogenetic and Serologic Markers

The serologic marker most strongly associated with iridocyclitis is the

presence of antinuclear antibodies. [1-3] Antinuclear antibodies are

present in 65% to 88% of these children. [9,10] They are usually present

in low titer and are of unknown specificity.

Rheumatoid factor is not usually present in children with iridocyclitis.

Rheumatoid factor positivity is most commonly seen in older children

with polyarticular disease that is clinically similar to adult

rheumatoid arthritis. Genetic factors may predispose to the development

of iridocyclitis. Recent data show that HLA-DR5 is correlated with the

presence of eye disease, and HLA-DR1 with its absence; HLA-DRw8, which

strongly predisposes to pauciarticular-onset JRA, was neutral with

respect to eye disease.

Clinical Characteristics of Iridocyclitis

The ocular inflammation is insidious in onset and asymptomatic in the

majority of patients. [1-3,5] Due to the lack of symptoms, the exact

time of onset of ocular involvement is frequently difficult to

determine. This emphasizes the need for slitlamp examination by an

ophthalmologist. Signs or symptoms in children with iridocyclitis may

include red eyes, decreased vision, unequal pupils, ocular pain, and

headaches. Most cases of iridocyclitis are bilateral; children with

unilaterial iridocyclitis may progress to bilateral iridocyclitis after

the initial 12 months of eye disease. Visual prognosis is improved by

early detection of iridocyclitis.

In 25% of iridocyclitis cases, the prognosis is very good. Twenty-five

percent of children respond poorly to treatment and may require surgery

for cataracts and/or glaucoma. This group is at risk for loss of vision

and may experience more ocular than articular morbidity. Approximately

50% of patients require prolonged treatment for moderate to severe

chronic inflammation; however, the visual prognosis in these patients is

generally good. Functional blindness has been reported in 15% to 40% of

affected eyes.

GUIDELINES FOR THE FREQUENCY OF OPHTHALMOLOGIC EXAMINATIONS IN CHILDREN

WITH

JUVENILE RHEUMATOID ARTHRITIS

The suggested frequency of ophthalmologic visits for children with JRA

without known iridocyclitis is presented in the Table. Once

iridocyclitis is diagnosed, the treating ophthalmologist will determine

the frequency of visits.

The subtype of juvenile arthritis is determined by the systemic features

of the illness and the number of joints with arthritis during the first

6 weeks of the illness. Pauciarticular JRA is defined by involvement of

four or fewer joints, polyarticular JRA is defined by involvement of

more than four joints, and systemic JRA is defined by a characteristic

rash associated with spiking fevers during the first 6 weeks of the

illness. Initial referral to an ophthalmologist should be made at the

time of diagnosis of JRA.

REFERENCES

1. Rosenberg AM. Uveitis associated with juvenile rheumatoid arthritis.

Semin Arthritis Rheum. 1987;16:158-173

2. Cassidy JT, Petty RE. Juvenile rheumatoid arthritis, special

considerations. In: Textbook of Pediatric Rheumatology. 2nd ed. New

York, NY: Churchill Livingstone; 1990:186-197

3. Petty RE. Current knowledge of the etiology and pathogenesis of

chronic uveitis accompanying juvenile rheumatoid arthritis. Rheum Dis

Clin North Am. 1987;13:19-36

4. Chylack LT Jr, Beinfang DC, Bellows AR, et al. Ocular manifestations

of juvenile rheumatoid arthritis. Am J Ophthalmol. 1975;79:1026-1033

5. Cassidy JT, Sullivan DB, Petty RE. Clinical patterns of chronic

iridocyclitis in children with juvenile rheumatoid arthritis. Arthritis

Rheum. 1977;20(suppl):224-227

6. Wolf MD, Lichter PR, Ragsdale CG. Prognostic factors in the uveitis

of juvenile rheumatoid arthritis. Ophthalmology. 1987;95:1242-1247

7. Brewer EJ, Bass J, Baum J, et al. Current proposed revision of JRA

criteria. Arthritis Rheum. 1977;20:195-199

8. Rosenberg AM, Oen KG. The relationship between ocular and articular

disease activity in children with juvenile rheumatoid arthritis and

associated uveitis. Arthritis Rheum. 1986;29:797-800

9. Petty RE, Cassidy JT, Sullivan DM. Clinical correlates of antinuclear

antibodies in juvenile rheumatoid arthritis. J Pediatr. 1973;83:386

10. Schaller JG, GD, Holborrow EJ, et al. The association of

antinuclear antibodies with the chronic iridocyclitis of juvenile

rheumatoid arthritis (Still's disease). Arthritis Rheum. 1974;17:409

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The recommendations in this statement do not indicate an exclusive

course of treatment or serve as a standard of medical care. Variations,

taking into account individual circumstances, may be appropriate.

PEDIATRICS (ISSN 0031 4005). Copyright © 1993 by the American Academy

of Pediatrics.

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without prior written permission from the American Academy of Pediatrics

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