Patterns and complications of uveitis among patients with Juvenile Idiopathic Arthritis

 It is the most common rheumatic disease of childhood. (2) There is a wide geographical variation regarding its prevalence, which ranges 7-400/100,000. (3) The diagnosis of JIA is mainly clinical. However, raised erythrocyte sedimentation rate, elevated C-reactive protein, negative rheumatoid factor (RF) and high anti-nuclear antibody titer (ANA) are frequently observed among JIA patients. (4)

Pediatric uveitis accounts for about 10% of uveitis cases. (5) JIA-associated uveitis occurs in 4-38% of JIA patients and accounts for 15-67% of pediatric uveitis. (2,4) Studies have reported that female gender, early age of onset, positive ANA and oligoarticular category of JIA are risk factors for developing uveitis in JIA patients. (5,6)

JIA-associated uveitis is usually asymptomatic, and children are incapable of providing accurate description of uveitis symptoms unless they face severe complications. Therefore, late diagnosis of uveitis may contribute to a high rate of ocular complications, which may cause visual impairment and blindness. (7) Ocular complications that may adversely affect the vision include band keratopathy, posterior synechiae, cataract, glaucoma, hypotony, macular edema, epiretinal membrane, and optic disc edema. (8)

The aim of this study is to explore the frequency and types of uveitis among JIA patients and identify uveitis complications and its impacts on vision.

 Method

        This was a prospective study conducted at the ophthalmology clinic of Queen Rania Al Abdullah Children Hospital between January 2017 and January 2018. All patients diagnosed with JIA were included in the study. The patients were screened for uveitis at the ophthalmology clinic. Patients with ocular diseases and complications not related to uveitis like congenital or steroid induced cataract and glaucoma were excluded from the study. Initially, data were obtained regarding age, gender, type and duration of JIA, type and duration of uveitis, ANA status, and treatment used. Detailed ocular examination including best corrected visual acuity using Snellen chart, anterior segment examination using slit lamp, posterior segment examination using 90D and 78D Volk lens and intra ocular pressure measurement using Goldmann applanation tonometry. The obtained data were analyzed and compared with the findings of other studies conducted in regional countries and worldwide.

Results

       A total of 54 children aged between 3 and 15 years (mean 9.4±2.4 years) were diagnosed with JIA at Queen Rania Al Abdullah Children Hospital. Of them, 74% were female (male to female ratio 1:3). The duration of JIA ranged from 6 months to 12 years (mean 5.1 years±1.9 years). The most common type of JIA was oligoarticular type (67%), followed by polyarticular (22%) and systemic onset types (11%). High ANA titer was noted in 70% of patients, and RF was detected in 7% of patients.

Table I : represents a comparison between JIA patients who developed uveitis and those who did not.

Table II: represents the best corrected visual acuity among eyes with JIA associated uveitis.

The risk factors for moderate to severe visual impairment were long duration of uveitis, late referral, delay in starting systemic treatment including the biologics, and glaucoma. Age and gender did not show any significant association with deterioration of vision.

Uveitis complications were reported in 16 eyes (64%), and Table III represents the types of complications encountered in those patients.

The most common complication responsible for visual impairment in JIA patients was cataract, followed by band keratopathy. However, glaucoma was most commonly associated with severe visual impairment. The most common form of uveitis was chronic anterior uveitis (68%), followed by recurrent anterior uveitis (24%) and pan uveitis (8%).

 Discussion

       Uveitis is the most common extra articular manifestation of JIA. It can result in serious ocular morbidities due to the high rate of associated complications (9). Although the prevalence of JIA is not yet studied in Jordan, JIA is commonly seen in pediatric medical practice. In our study, the mean age of the patients was 9.4 years and the mean duration of JIA was 4.9 years. In addition, the mean age of JIA onset worldwide has a wide range of variability; it can be as low as 2.8 years in the USA to as high as 7.3 years in Switzerland (10,11). This variability could be implicated by genetic factors. In the present study, the mean age of JIA onset was 4.3 years.

silent and asymptomatic disease in most of the JIA patients. Moreover, patients will not attend the ophthalmology clinic unless there is significant decline in vision (15). In Jordan, there is no routine or programmed ocular examination for preschool and school children. This may hinder the detection of uveitis in cases that may precede the onset of arthritis.

Visual impairments are commonly seen in JIA-associated uveitis due to the high rate of ocular complications resulting from uveitis, which may approach 73% (18). In the present study, ocular complications were detected in 64% of eyes, and visual impairment were seen in only 32% of eyes. These rates were lower than the global values. Only 16% of eyes showed moderate to severe visual impairment (≤0.3) and 8% eyes showed visual acuity of less than 0.1. In comparison, Gregory et al reported that 40% of eyes had visual acuity of 20/50 or less and 24% of eyes were found to have visual acuity of 20/200 or less (19). The policy at Queen Rania Al Abdullah Children Hospital is to immediately start systemic immune modulating therapy, including biologics for JIA patients with chronic uveitis, which might be the main reason for those lower rates. Risk factors for moderate to severe visual impairments were long duration of uveitis, late referral, delay in starting systemic treatment including the biologics, and glaucoma. So, 3 out of 4 of the risk factors are modifiable and adjustable. Therefore, a new policy should be adopted, particularly in peripheral hospitals, to control those risk factors before significant visual impairment takes place. These results are in line with other studies, except for gender, as age and gender did not show any significant association with deterioration of vision. Meanwhile, Edelsten et al. reported a higher rate of complications and visual impairment among males compared to females (19,20).

Uveitis complications were seen in 64% of eyes. This rate is higher than the rates in adults (21). This is attributed to the nature of uveitis, i.e., chronic and asymptomatic, among JIA patients (white eye). In addition, children are not aware and are unable to express their complaints related to uveitis.

The most common complications seen in JIA uveitis patients were cataract, glaucoma, and band keratopathy. However, glaucoma was associated with a higher risk for severe visual impairment. Glaucoma may develop in those patients as a result of damage to the trabecular meshwork drainage system or because of the steroids used to treat uveitis. In addition, angle closure resulting from anterior or posterior synechiae may also lead to glaucoma.

Finally, the present study is the first in Jordan to describe the frequency of uveitis among JIA patients and the rates of ocular complications and visual impairments seen in those patients. The sample size was relatively small, but higher frequency of uveitis with lower rates of moderate to severe visual impairments and ocular complications were seen in JIA patients at Queen Rania Al Abdullah children hospital compared to studies conducted in the USA and Europe. Genetic and geographical factors might be implicated in this issue. More comprehensive data should be obtained from other medical sectors to calculate more accurate rates and prevalence. In addition, this study showed that most of the risk factors for moderate to severe visual impairments are controllable, so effective measures should be activated to reduce visual morbidities.

Conclusions

      Higher frequency of uveitis with lower rates of moderate to severe visual impairments and ocular complications were seen in JIA patients. Long duration of uveitis, late referral, delay in starting systemic treatment including the biologics, and glaucoma were associated with moderate to severe visual impairment. The most common complications seen in JIA uveitis patients were cataract, glaucoma and band keratopathy.

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