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