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Clinical Profile of Juvenile Idiopathic Arthritis Associated Uveitis in A ...

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UVEA SESSION<br />

715<br />

<strong>Cl<strong>in</strong>ical</strong> <strong>Pr<strong>of</strong>ile</strong> <strong>of</strong> <strong>Juvenile</strong> <strong>Idiopathic</strong> <strong>Arthritis</strong> <strong>Associated</strong> <strong>Uveitis</strong><br />

<strong>in</strong> A Tertiary Care Eye Centre<br />

Dr. Anushree V. Kaduskar, Dr. Amala Elizabeth George, Dr. S. Sudharshan,<br />

Dr. Jyotirmoy Biswas<br />

(Present<strong>in</strong>g Author: Dr. Anushree V. Kaduskar)<br />

<strong>Juvenile</strong> idiopathic arthritis (JIA) is def<strong>in</strong>ed as<br />

cl<strong>in</strong>ical evidence <strong>of</strong> chronic arthritis (with a<br />

duration <strong>of</strong> at least 3 months) <strong>of</strong> unknown cause<br />

<strong>in</strong> a child younger than 16 years <strong>of</strong> age.3<br />

Approximately 6% <strong>of</strong> all uveitis occur <strong>in</strong><br />

children. 1 <strong>Uveitis</strong> associated with juvenile


716 AIOC 2010 PROCEEDINGS<br />

idiopathic arthritis is the most common cause <strong>of</strong><br />

chronic <strong>in</strong>traocular <strong>in</strong>flammation among<br />

paediatric population. 2 We describe the cl<strong>in</strong>ical<br />

pr<strong>of</strong>ile, complications, management and visual<br />

outcomes <strong>in</strong> patients with JIA associated uveitis<br />

<strong>in</strong> a tertiary eye care centre <strong>in</strong> India.<br />

Materials and Methods<br />

This is a retrospective study <strong>in</strong> which we<br />

reviewed the records <strong>of</strong> 40 patients (80 eyes) who<br />

presented to our uveitis referral centre between<br />

1994 and 2008.Complete eye evaluation was<br />

done on each visit and <strong>in</strong>cluded a complete<br />

history, visual acuity, cycloplegic refraction, slit<br />

lamp exam<strong>in</strong>ation <strong>in</strong>traocular pressure<br />

estimation and dilated fundoscopic exam<strong>in</strong>ation.<br />

The Standardization <strong>of</strong> <strong>Uveitis</strong> Nomenclature<br />

(SUN) Work<strong>in</strong>g Group criteria were used to<br />

report cl<strong>in</strong>ical data. Results <strong>of</strong> laboratory<br />

<strong>in</strong>vestigations, diagnostic and therapeutic<br />

procedures were analyzed. The systemic<br />

condition <strong>of</strong> the patient was managed by a<br />

rheumatologist.<br />

Results<br />

Forty patients (80 eyes) with JIA seen at our<br />

hospital between 1994 and 2008 were analyzed <strong>in</strong><br />

the study. Age at presentation at the hospital<br />

ranged between 3 to 24 years (mean <strong>of</strong> 12.27+/-<br />

6.48 years). The male: female ratio was 3:2.Thirty<br />

one patients (77.5%) had bilateral presentation<br />

and seven (17.5%) had unilateral presentation.<br />

There was no ocular <strong>in</strong>volvement <strong>in</strong> 2 patients at<br />

presentation and on subsequent follow up. The<br />

age <strong>of</strong> onset <strong>of</strong> arthritis ranged from 1.5-16 years<br />

(mean <strong>of</strong> 7.42+/-4.2 years) and the age <strong>of</strong> onset<br />

<strong>of</strong> uveitis ranged from 3-24 years (mean <strong>of</strong><br />

9.08+/-5.5 years).Onset <strong>of</strong> arthritis preceded the<br />

onset <strong>of</strong> uveitis <strong>in</strong> thirty seven patients (92.5%),<br />

uveitis was the primary manifestation <strong>in</strong> 1<br />

patient and 2 patients had only arthritis. The<br />

presentation <strong>of</strong> arthritis was pauciarticular <strong>in</strong> 32<br />

patients (80%) and polyarticular <strong>in</strong> 6 patients<br />

(15%). Knee jo<strong>in</strong>t was the most commonly<br />

<strong>in</strong>volved jo<strong>in</strong>t <strong>in</strong> 22 patients(55%). Ankle jo<strong>in</strong>t<br />

was <strong>in</strong>volved <strong>in</strong> 9 patients, wrist jo<strong>in</strong>t <strong>in</strong> 5<br />

patients, small jo<strong>in</strong>ts <strong>in</strong> 3 patients and elbow jo<strong>in</strong>t<br />

<strong>in</strong> 2 patients. Dim<strong>in</strong>ution <strong>of</strong> vision was the most<br />

common symptom <strong>in</strong> 56 eyes followed by<br />

redness <strong>in</strong> 26 eyes. Other present<strong>in</strong>g symptoms<br />

<strong>in</strong>cluded pa<strong>in</strong> <strong>in</strong> 15 eyes, photophobia <strong>in</strong> 12 eyes<br />

and floaters <strong>in</strong> 6 eyes. Seven eyes had no<br />

perception <strong>of</strong> light at presentation. The most<br />

common type <strong>of</strong> uveitis <strong>in</strong> our series was chronic<br />

anterior uveitis <strong>in</strong> 60 eyes (75%) followed by<br />

<strong>in</strong>termediate uveitis <strong>in</strong> 10 eyes (12.5%) and<br />

panuveitis <strong>in</strong> 2 eyes (2.5%). Posterior uveitis was<br />

the presentation <strong>in</strong> 5 eyes. Acute anterior uveitis<br />

with hypopyon was seen <strong>in</strong> 1 eye. Band shaped<br />

keratopathy was present <strong>in</strong> 34 eyes<br />

(42.5%),complicated cataract <strong>in</strong> 33(41.25%) and<br />

seclusio papillae <strong>in</strong> 34(42.5%). Other features<br />

seen were aphakia <strong>in</strong> 3 eyes(3.75%),<br />

pseudophakia <strong>in</strong> 4 eyes(10%) and iris nodules <strong>in</strong><br />

1 eye. Posterior segment features seen were pars<br />

planitis and glaucomatous optic atrophy <strong>in</strong> 1 eye<br />

each (1.25%), cystoid macular oedema and total<br />

ret<strong>in</strong>al detachment <strong>in</strong> 2 eyes each(2.5%) and disc<br />

hyperemia <strong>in</strong> 5 eyes(6.25%). Fundus exam<strong>in</strong>ation<br />

was normal <strong>in</strong> 30 eyes.30 patients were<br />

<strong>in</strong>vestigated for rheumatoid arthritis (RA) factor<br />

and 3(10%) amongst them tested positive for the<br />

same. Anti nuclear antibodies (ANA) were<br />

positive <strong>in</strong> 7 patients (26.9%) out <strong>of</strong> 26 patients<br />

who were tested. All patients were tested for<br />

erythrocyte sedimentation rate (ESR).Sixteen<br />

patients had raised ESR. One young adolescent<br />

male was positive for HLA B 27. Prednisolone<br />

acetate 1% eye drops were used <strong>in</strong> taper<strong>in</strong>g<br />

dosage and the frequency <strong>of</strong> application was<br />

based on the severity <strong>of</strong> anterior chamber<br />

reaction. A comb<strong>in</strong>ation <strong>of</strong> topical and oral<br />

steroids was used <strong>in</strong> 12 patients(30%).Topical,<br />

periocular and oral steroids were used <strong>in</strong> 8<br />

patients(20%). 2 patients required <strong>in</strong>travenous<br />

(methyl prednisolone) and <strong>in</strong>travitreal steroids<br />

each. Oral steroids were used <strong>in</strong> the dose <strong>of</strong><br />


UVEA SESSION<br />

717<br />

ethylenediam<strong>in</strong>etetraacetic acid (EDTA) for BSK<br />

was done <strong>in</strong> 6 eyes (7.5%).Other procedures<br />

<strong>in</strong>cluded vitrectomy and belt buckle <strong>in</strong> 4 eyes<br />

each and trabeculectomy <strong>in</strong> 1 eye. Five eyes<br />

(6.25%) required YAG laser capsulotomy for<br />

posterior capsular opacification follow<strong>in</strong>g<br />

<strong>in</strong>traocular lens implantation. IOL removal with<br />

vitrectomy was done <strong>in</strong> 4 eyes due to severe post<br />

operative reactivation <strong>of</strong> uveitis and IOL<br />

<strong>in</strong>tolerance after surgery.11 patients had<br />

recurrence <strong>of</strong> <strong>in</strong>fection amongst which 3 patients<br />

were not on any medications at the time <strong>of</strong><br />

recurrence. Visual acuity at f<strong>in</strong>al follow up<br />

showed improvement <strong>in</strong> 31eyes (38.75%), was<br />

stable <strong>in</strong> 34 eyes (42.5%) and deteriorated <strong>in</strong> 15<br />

eyes (18.75%) despite treatment due to<br />

complications <strong>of</strong> JIA.<br />

Discussion<br />

JIA associated uveitis is reported to occur most<br />

commonly <strong>in</strong> young girls seropositive for ANA<br />

with pauciarticular-onset arthritis.4,5In our<br />

study, males outnumbered females <strong>in</strong> contrast to<br />

studies <strong>in</strong> western population where females<br />

were more commonly affected.2,5Sabri et al<br />

reported JIA uveitis to occur <strong>in</strong> 61% <strong>of</strong> patients<br />

who had pauciarticular JIA. Pauciarticular type<br />

<strong>of</strong> JIA was seen <strong>in</strong> 80% <strong>of</strong> our patients. The<br />

reason for <strong>in</strong>creased <strong>in</strong>cidence <strong>of</strong> pauciarticular<br />

JIA <strong>in</strong> our series is because patients with ocular<br />

symptomatology are seen by us and<br />

pauciarticular type JIA is most commonly<br />

associated with uveitis.2,7 The mean time from<br />

diagnosis <strong>of</strong> JIA to diagnosis <strong>of</strong> uveitis was 1.66<br />

years. Chalom et al9 have reported that a shorter<br />

<strong>in</strong>terval between diagnosis <strong>of</strong> JIA and uveitis was<br />

a significant risk factor for develop<strong>in</strong>g uveitic<br />

complications. The most common type <strong>of</strong> uveitis<br />

<strong>in</strong> our series was chronic anterior uveitis as <strong>in</strong><br />

1. O’Brien JM,Albert DM,Foster CS.<strong>Juvenile</strong><br />

rheumatoid arthritis. In: Albert DM,Jackobiec<br />

FA.eds.Pr<strong>in</strong>ciples and practice <strong>of</strong> ophthalmology:<br />

<strong>Cl<strong>in</strong>ical</strong> practice. Vol.5. Philadelphia: W.B.Saunders<br />

Co,1994;chap233<br />

2. Kanski JJ.<strong>Uveitis</strong> <strong>in</strong> juvenile chronic arthritis:<br />

<strong>in</strong>cidence, cl<strong>in</strong>ical features and prognosis. EYE<br />

1988;2:641-5.<br />

3. Petty RE,Southwood TR, Manners P,Baum J,Glass<br />

DN,Goldenberg J,et al.International league <strong>of</strong><br />

Associations for Rheumatology classification <strong>of</strong><br />

References<br />

other studies.2,7Similar to other studies cataracts<br />

(41.25%) and BSK (42.5%) were the most common<br />

complications noted <strong>in</strong> our cohort.5We found<br />

ANA positivity <strong>in</strong> only 7 <strong>of</strong> the 26 patients tested<br />

for the same. Western studies4 have noted a<br />

higher <strong>in</strong>cidence and the risk <strong>of</strong> development <strong>of</strong><br />

severe disease <strong>in</strong> females with ANA positivity.<br />

Agarwal et al.10 have however noted the rarity<br />

<strong>of</strong> ANA positivity <strong>in</strong> Indian population.<br />

Methotrexate was the most commonly used<br />

immunosuppressant <strong>in</strong> 19 patients (47.5%) <strong>in</strong> our<br />

study and showed good results <strong>in</strong> concordance<br />

with study by Hemady et al.11We found<br />

methotrexate as an effective steroid spar<strong>in</strong>g agent<br />

especially as a long term option. Liver function<br />

tests and blood counts <strong>of</strong> the patients on<br />

methotrexate have to be monitored periodically<br />

(atleast every month) <strong>in</strong> association with an<br />

<strong>in</strong>ternist. Kanski 1had noted good results after<br />

lensectomy for cataracts <strong>in</strong> patients with JIA. In<br />

our study, cataract was the most common cause<br />

for which patients underwent surgery. Most <strong>of</strong><br />

the patients who underwent lensectomy were<br />

rehabilitated with aphakic correction with<br />

spectacles or contact lenses.<br />

Our study <strong>in</strong>dicates that <strong>in</strong> India, JIA associated<br />

uveitis commonly presents as pauciarticular type<br />

and is more common <strong>in</strong> males. Investigations for<br />

RA and ANA are probably not <strong>of</strong> much help <strong>in</strong><br />

diagnosis or prognosis <strong>of</strong> these patients.<br />

Complications such as BSK and complicated<br />

cataract need prompt treatment for help<strong>in</strong>g<br />

speedy visual recovery. As long-term treatment<br />

options, immunosuppressives are a better choice<br />

than steroids. These patients need to be followed<br />

up for long periods as recurrence <strong>of</strong> uveitis,<br />

development <strong>of</strong> complications like glaucomatous<br />

optic atrophy and hypotony can occur <strong>in</strong> such<br />

patients.<br />

juvenile idiopathic arthritis.Second revision.<br />

Edmonton, 2001. J Rheumatol 200$;31:390-92.<br />

4. Dana MR, Merayo-Lloves J, Schaumberg DA, Foster<br />

CS. Visual outcomes prognosticators <strong>in</strong> juvenile<br />

rheumatoid arthritis-associated uveitis.<br />

Ophthalmology 1997;104:236-44.<br />

5. Kourosh Sabri,Rotraud KS,Earl DS,Alex VL.Course,<br />

complications, and outcome <strong>of</strong> juvenile arthritisrelated<br />

uveitis. J APPOS 2008;12:539-45.<br />

6. Kodsi SR, Rub<strong>in</strong> SE, Milojevic D, Ilowite N, Gottlieb<br />

B. Time <strong>of</strong> onset <strong>of</strong> uveitis <strong>in</strong> children with juvenile


718 AIOC 2010 PROCEEDINGS<br />

rheumatoid arthritis. J AAPOS 2002;6:373-6.<br />

7. Kanski JJ. <strong>Juvenile</strong> arthritis and uveitis. Surv<br />

Ophthalmol 1990;34:253-67.<br />

8. Jabs DA, Nusssenblatt RB, Rosenbaum JT.<br />

Standardization <strong>of</strong> uveitis nomenclature for<br />

report<strong>in</strong>g cl<strong>in</strong>ical data: Results <strong>of</strong> the First<br />

International Workshop. Am J Ophthalmol<br />

2005;140:509-16.<br />

9. Chalom EC,Goldsmith DP,Kohler MA,Bittar B,Rose<br />

CD,Ostrov BE,et al.Prevalence and outcome <strong>of</strong><br />

uveitis <strong>in</strong> a regional cohort <strong>of</strong> patients with juvenile<br />

rheumatoid arthritis.J Rheumatol 1997;24:2031-4.<br />

10. Aggarwal A, Misra RN. <strong>Juvenile</strong> rheumatoid<br />

arthritis <strong>in</strong> India-rarity <strong>of</strong> ant<strong>in</strong>uclear antibody and<br />

uveitis. Indian J Pediatr 1996;63:301-4.<br />

11. Hemady RK, Baer JC, Foster CS, Foster CS.<br />

Immunosuppressive drugs <strong>in</strong> the management <strong>of</strong><br />

progressive, corticosteroid-resistant uveitis<br />

associated with juvenile rheumatoid arthritis. Int<br />

Ophthalmol Cl<strong>in</strong> 1992;32:241-52.

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