Demographic and Clinical Features of Pediatric Uveitis at a Tertiary Referral Center in Iran.

PURPOSE: To determine the clinical features and distribution of uveitis in the pediatric age group at a referral eye care center in Shiraz, South Iran.
MATERIALS AND METHODS: All new cases of uveitis in patients 18-year-old or younger referred from January 2007 to December 2013 were enrolled in this study. The patient underwent a complete history of systemic and ocular diseases, comprehensive ophthalmic examination. Patients were classified according to the International Uveitis Study Group definitions. The definitive diagnosis was based on clinical manifestations and laboratory investigations.
RESULTS: Seventy-seven eyes (54 patients) comprised the study sample. The mean age at the onset of uveitis was 12.5 years. The female-to-male ratio was 1.25. Anterior uveitis was the most frequent anatomical location (40.7%), followed by intermediate uveitis (33.3%), posterior uveitis (18.5%), and panuveitis (7.5%). Seventy-four percent of patients presented with chronic uveitis. Noninfectious uveitis (81.5%) was the most frequent etiology. Thirty-seven percent of patients had a specific diagnosis for uveitis, and 63% were classified as idiopathic cases. Toxocariasis was the most common infectious cause. Associated systemic diseases were present in 14.8% of patients. The most frequent systemic disease was juvenile idiopathic arthritis in 9.2% of patients. Complications occurred in 66 (85.5%) of affected eyes. The most common complications were posterior synechia (20.7%), cataract (18.8%), and cystoid macular edema (12.9%).
CONCLUSIONS: Uveitis in the majority of children had an insidious onset and was chronic. Over half the patients had a specific diagnosis. Idiopathic cases were more common in the intermediate uveitis group.

INTRODUCTION

Uveitis is the third leading cause of blindness worldwide.12 Uveitis is more common in adults; however, children comprise 2.2–21.6% of patients in various studies.12345

The lack of significant symptoms and complaints from children lead to a late referral to ophthalmologist and often disease is in its advanced stage with severe visual morbidity. Several authors have published the pattern of uveitis in childhood in their geographical areas. In our survey on the pattern of uveitis in Iran, 9.4% of uveitis patients were in the pediatric age group.6 To categorize the pattern of uveitis in children, we retrospectively reviewed the records of patients over a 6-year period at a uveitis clinic in a referral eye center in Shiraz, South Iran.

MATERIALS AND METHODS

A retrospective chart review was performed all new consecutive cases under the age of 18 years who presented with uveitis between January 2007 and December 2013 to the Ocular Inflammation and Uveitis Service, Department of Ophthalmology, Shiraz University of Medical Sciences, Shiraz, Iran. Patients were excluded if they had postoperative complications, postsurgical inflammation, and/or ocular trauma. The research protocol adhered to the tenets of the Declaration of Helsinki and was approved by the ethics committee of Shiraz University of Medical Sciences.

A detailed standard uveitis questionnaire was completed for each case. A comprehensive ocular and systemic history were obtained for each patient, accompanied by the review of systems. Ocular examination included external eye examination, measurement of best-corrected Snellen visual acuity, slit lamp biomicroscopy, applanation tonometry, Marcus-Gunn pupil testing, dilated fundus examination, and in some cases, examination under anesthesia.

Some cases required additional workups such as fluorescein angiography, visual field testing, electrophysiology tests, or ophthalmic ultrasonography. If indicated, patients were referred to pediatricians, rheumatologists, or infectious disease specialists.

Uveitis was classified according to the International Uveitis Study Group criteria based on the anatomic location of the inflammation and categorized as anterior, intermediate, posterior, or panuveitis, and also disease description for clinical onset, duration, and course.7

Anterior uveitis was defined when there was only iritis and/or iridocyclitis. Intermediate uveitis was defined as inflammation confined to the middle aspect of the eye (posterior ciliary body, pars plana). Posterior uveitis was defined as intraocular inflammation primarily involving the retina and/or choroid. Panuveitis was defined as inflammation in the anterior chamber, vitreous, and retina, or choroid.

Based on the clinical course, uveitis was classified as: Acute uveitis if there was sudden onset inflammation lasting for <3 months; chronic uveitis if the duration of inflammation was >3 months; and recurrent uveitis when repeated episodes of intraocular inflammation were separated by periods of inactivity without treatment for ≥3 months.

Each patient underwent routine laboratory evaluation including complete blood count, white blood count with differentials, erythrocyte sedimentation rate, C-reactive protein, blood urea nitrogen with electrolytes, and urinalysis. Where indicated, specific tests such as antinuclear antibodies, specific serology test, and imaging were ordered.

Uveitis was classified as (1) idiopathic if all ocular and systemic evaluations and (2) workup indicated no specific ocular or systemic condition.

RESULTS

The demographic and clinical characteristics of subjects with pediatric uveitis are presented in Table 1. Seventy-seven eyes of 54 children were enrolled in this study. The mean age at presentation was12.5 ± 5 years (range, 2–18 years). Thirty (55.5%) patients were female and 24 patients (44.5%) were male, with female-to-male ratio of 1.25. Thirty-one (57.5%) patients had unilateral uveitis, and 23 (42.5%) had bilateral uveitis. Fourteen (26%) patients presented with an acute clinical presentation and 40 (74%) had a chronic clinical course. The mean best-corrected visual acuity (BCVA) at presentation was 20/60 (6/18; −0.5 LogMar) and ranged from hand motion (−3.0 LogMar) to 20/20 (6/6; 0 LogMar). BCVA was >20/40 (−0.3 LogMar) in 32 (41.5%) eyes and worse than 20/200 (−1.0 LogMar) in 15 (19.5%) eyes.

Table 1

General features of the patients with pediatric uveitis

A specific diagnosis was achieved in 20 (37%) patients [Table 2], and the uveitis is deemed there were 34 (63%) patients diagnosed with idiopathic uveitis. The majority of idiopathic cases were in the intermediate uveitis group (17 of 18 cases, 94.4%), followed by anterior uveitis (13 of 22 cases, 60%), panuveitis (2 of 4 cases, 50%), and posterior uveitis (2 of 10 cases, 20%). There were 10 (18.5%) patients with infectious uveitis and 44 (81.5%) patients with noninfectious etiology. Anterior uveitis was the most common type (22 patients, 40.7%), followed by intermediate uveitis (18 patients, 33.3%), posterior uveitis (10 patients, 18.5%), and panuveitis (4 patients, 7.5%).

Table 2

Classification of the diagnosis of uveitis in pediatric cases

Eight patients (14.8%) had associated systemic disease. The most common systemic disease was juvenile idiopathic arthritis (JIA) in 5 (9.2%) patients. Other associated systemic diseases included Vogt–Koyanagi–Harada syndrome (one patient, 1.85%), acute lymphoid leukemia-L2 as masquerading disease (one patient, 1.85%), and sarcoidosis (one patient, 1.85%).

Toxocariasis in four patients (7.4%) and toxoplasmosis in another four patients (7.4%) were the most common infectious etiologies in both posterior uveitis (80%) and all patients (14.8%).

Table 3 presents ocular complications of pediatric uveitis. Complications occurred in 66 (85.5%) of affected eyes. The most common complications were posterior synechia, in 16 of 77 eyes (20.7%), cataract in 14 eyes (18.8%), and cystoid macular edema (CME) in 10 eyes (12.9%). Posterior synechia was more common in patients with anterior uveitis, and cataract and CME were more common in patients with intermediate uveitis.

Table 3

Ocular complications in pediatric uveitis

DISCUSSION

Our previous study of uveitis at a tertiary referral center indicated that children account for 9.4% of patients.6 In our study, the mean age at diagnosis was 12.5 years while it was between 8 and 10 years of age (range, 1–16 years) in other studies.458 We found a slightly greater preponderance of females with uveitis than males, which concurs with previous studies.9101112

Although childhood patients usually have bilateral uveitis;13458 in the present study, uveitis was unilateral in almost the half of the patients. We found anterior uveitis was the most common, accounting for 40.7%, followed by intermediate uveitis 33.3%, posterior uveitis 18.5%, and panuveitis 7.5%. This outcome is similar to the previous studies that reported anterior uveitis (30–58.4%) was the most common type of uveitis in children, followed by posterior uveitis (13.8–50%), intermediate uveitis (10–27.7%), and panuveitis (5–21%).511121314

The diagnosis of uveitis in children varies based on geographic location in relation to anatomical distribution, infectious versus noninfectious etiologies, and the frequency of idiopathic cases. In our study, 20 (37%) patients had uveitis that had a specific diagnosis, and 34 (63%) patients had idiopathic uveitis. The largest number of idiopathic cases occurred in the intermediate uveitis group (94.4%), followed by anterior uveitis (59%), panuveitis (50%), and posterior uveitis (33.3%).

Systemic associations were found in 8 (14.8%) patients. The most common systemic disease was JIA, accounting for 9.2% of all cases.

This association is much higher in most published studies from the Western countries which have identified JIA-associated anterior uveitis as the most frequent type of anterior uveitis in children (67–77%).3515 However, studies from the Mediterranean region and North Africa report that the frequency of JIA-associated anterior uveitis was similar to our study, accounting for 6.25–15% of all pediatric uveitis cases.191416

The most common specific diagnosis in our series was JIA (9.2%), toxoplasmosis (7.4%), and toxocariasis (7.4%). Toxoplasmosis was responsible for only 2–10% of all cases in pediatric patients from the Western countries.3511 Studies from the Middle East and India reported a much higher prevalence of toxoplasmosis in children.141718 An Indian survey discovered that infectious uveitis is more common in children than in adults.17

Seventy-four percent of affected children in the current survey had a chronic course with insidious onset. Other studies have reported chronic uveitis in up to 87% of affected children.58

Complications occurred in the large majority of (85.5%) affected eyes. Common complications were posterior synechia (20.7% of eyes), cataract (18.8% of eyes), and CME (12.9% of eyes). Posterior synechia was more frequent in patients with anterior uveitis whereas cataract and CME were more common among intermediate uveitis cases. The chronic course of many diseases in pediatric uveitis and the higher rate of complications lead to significant visual loss and morbidity. Vision loss is more common in pediatric uveitis than in adult uveitis, despite a lower incidence of uveitis in children.19 In our study, BCVA was >20/40 (−0.3 LogMar) in 32 (41.5%) eyes, and it was worse than 20/200 (−1.0 LogMar) in 15 (19.5%) eyes. The worse visual prognosis was associated with severe JIA-associated uveitis and was comparable to some reports.520

CONCLUSIONS

This study revealed that although uveitis is not as common in the pediatric age group as in adults, if not diagnosed and treated properly, can have devastating complications resulting in severe vision loss. Uveitis in the majority of children had an insidious onset and was chronic. Over half the patients had a specific diagnosis. Idiopathic cases were more common in the intermediate uveitis group.

Financial support and sponsorship

This study was supported by a grant from Shiraz University of Medical Sciences, Grant No: 1390-3816.

Conflicts of interest

There are no conflicts of interest.