|Year : 2017 | Volume
| Issue : 2 | Page : 31-34
Study of the different ocular manifestations in children with juvenile idiopathic arthritis
Eiman M Abd El Latif MD 1, Ghada F El Derini2
1 Department of Ophthalmology, Faculty of Medicine, Alexandria University, Alexandria, Egypt
2 Department of Paediatrics, Faculty of Medicine, Alexandria University, Alexandria, Egypt
|Date of Submission||12-Mar-2015|
|Date of Acceptance||23-Sep-2015|
|Date of Web Publication||20-Jul-2017|
Eiman M Abd El Latif
5 Soliman Mahmoud Street, Cleopatra, 2nd floor, Alexandria, 32124
Source of Support: None, Conflict of Interest: None
The aim of this study was to describe the features of uveitis in children with juvenile idiopathic arthritis (JIA).
Patients and methods
The present study was an observational study of 132 eyes of 75 children with JIA and uveitis, who were examined with a slit-lamp biomicroscope and a noncontact fundus lens. The features of uveitis are described.
Among the 75 patients with JIA-associated uveitis, 132 eyes were affected by uveitis. At least one ocular complication was present in 64% of patients and in 67% of eyes affected with uveitis at presentation. In the 132 affected eyes, band keratopathy was the most frequent complication observed (32%), followed by posterior synechiae (28%), cataract (22%), and ocular hypertension (15%).
In summary, poor vision and ocular complications still occur commonly among children with JIA-related uveitis, particularly if there is a long delay in referral to a tertiary-care center.
Keywords: anterior chamber cells, band keratopathy, juvenile idiopathic arthritis, posterior synechiae, uveitis
|How to cite this article:|
Abd El Latif EM, El Derini GF. Study of the different ocular manifestations in children with juvenile idiopathic arthritis. J Egypt Ophthalmol Soc 2017;110:31-4
|How to cite this URL:|
Abd El Latif EM, El Derini GF. Study of the different ocular manifestations in children with juvenile idiopathic arthritis. J Egypt Ophthalmol Soc [serial online] 2017 [cited 2018 Apr 20];110:31-4. Available from: http://www.jeos.eg.net/text.asp?2017/110/2/31/211139
| Introduction|| |
Juvenile idiopathic arthritis (JIA) is a group of arthritides of unknown etiology that begins before 16 years of age and persists for at least 6 weeks. It is the most common systemic disorder associated with uveitis in childhood, accounting for ∼75% of all pediatric anterior uveitis cases ,,. Risk factors for the development of uveitis in patients with JIA include female sex, oligoarticular arthritis, young age at onset of arthritis, antinuclear antibody (ANA) positivity, and rheumatoid factor negativity ,,. Uveitis is estimated to occur in ∼30% of patients who are ANA-positive regardless of whether they have persistent oligoarticular, extended oligoarticular, or rheumatoid factor-negative polyarticular arthritis . Typically, uveitis in these patients is a chronic, bilateral, nongranulomatous anterior form that is gradual in onset and asymptomatic. In the majority of cases, uveitis is diagnosed within 4 years of diagnosis of arthritis ,,.
Severe visual impairment has been reported in up to 40% of patients ,. Increased severity of ocular disease at the initial examination has been reported as a risk factor for poor visual acuity outcomes at the last follow-up visit, but the follow-up time in these series are variable ,. Other factors reported to be predictive for visual impairment during follow-up include uveitis onset before or at the time of arthritis diagnosis ,, a short duration between onset of arthritis and uveitis, and male sex .
Chronic intraocular inflammation can lead to structural damage of ocular tissue and subsequent vision loss. Long-term ocular complications of JIA-associated uveitis include cataract, band-shaped keratopathy, posterior synechiae, glaucoma, hypotony, and maculopathy. The degree of ocular disease at initial examination may be an important prognostic factor in terms of development of ocular complications and loss of visual acuity during follow-up. Risk factors for presenting with ‘severe’ uveitis have been reported as male sex and having uveitis diagnosed 6 months after the diagnosis of arthritis.
This study assessed the frequencies of different ocular manifestations at presentation, in 132 eyes of children with JIA-associated uveitis.
| Patients and methods|| |
This was an observational study.
A total of 132 eyes of 75 patients with JIA-associated uveitis who presented to us at El Shatby University Hospital and the Main University Hospital in Alexandria were included in this study. Arthritis was classified according to the American College of Rheumatology criteria for juvenile rheumatoid arthritis. The study has been approved by the ethical committee of Alexandria university and an informed consent was taken from every patient before enrollment in the study.
Ophthalmological examinations included intraocular pressure assessment, as well as findings from the slit-lamp and dilated fundus examinations at presentation.
Main outcome measures
Frequencies of ocular complications observed at presentation were assessed. Complications included presence of cataract, posterior synechiae, band-shaped keratopathy, ocular hypertension, hypotony, macular edema, and optic nerve head edema. Ocular hypertension was defined as intraocular pressure elevation greater than 21 mmHg, and hypotony was defined as intraocular pressure less than 5 mmHg.
Frequencies of variables were tabulated for patients and for affected eyes. All analyses were performed using SPSS, version 18. Data were analyzed using the statistical package for social sciences (version 20; SPSS Inc., Chicago, Illinois, USA).
| Results|| |
The median age at diagnosis of arthritis for all types of JIA was 5 years (range, 1–16 years), and among patients with the persistent oligoarticular type of JIA the median age at the time of diagnosis of arthritis was 4 years. The median age at the time of diagnosis of uveitis for all patients in the study population was 7 years (range, 1–18 years). Among patients with persistent oligoarthritis, the median age at diagnosis of uveitis was 5 years. The majority of patients were female (73%), had persistent oligoarticular arthritis (81%), and were ANA positive (70%).
In this study, 71% of patients had bilateral disease at presentation; 87% had anterior uveitis; and 13% had anterior and intermediate uveitis. Before presentation, 35% of patients had been treated with oral corticosteroids for their uveitis, 19% with methotrexate, and 4% with cyclosporine at some point during their disease course. At presentation, 12% of patients were taking systemic corticosteroids, 16% were taking methotrexate, and 3% were taking cyclosporine for their uveitis.
There was a considerable delay between the diagnosis of arthritis and the diagnosis of uveitis: 17% of the patients were diagnosed with arthritis and uveitis simultaneously, and 19% developed arthritis after the diagnosis of uveitis was made. Among patients who developed uveitis after the diagnosis of arthritis, the median time to diagnosis of uveitis was 6 months, and 75% of patients were diagnosed within 3 years of being diagnosed with arthritis.
Frequency of complications at presentation
Among the 75 patients with JIA-associated uveitis, 132 eyes were affected by uveitis. At least one ocular complication was present in 64% of patients and in 67% of eyes affected with uveitis at presentation. Among the 132 affected eyes, band keratopathy was the most frequent complication observed (32%), followed by posterior synechiae (28%), cataract (22%), and ocular hypertension (15%). Gonioscopy was performed in 15 patients (30 eyes) at presentation, and 17/30 (57%) eyes were found to have peripheral anterior synechiae. Less-frequent complications included hypotony (9%), epiretinal membrane (4%), optic nerve edema (4%), and macular edema (3%). Active intraocular inflammation, as evidenced by +0.5 anterior chamber cells, was observed in 44% of affected eyes at presentation. Anterior chamber flare of +1 was present in 49% of affected eyes. Active intraocular inflammation was associated with presence of at least one ocular complication at presentation (odds ratio, 2.88; 95% confidence interval, 1.40–5.92; P=0.005).
Risk factors for presenting with an ocular complication
The risk factors for presenting with at least one ocular complication in an eye with uveitis are summarized in [Table 1]. In the univariate analysis, younger age, bilateral uveitis, shorter duration between the diagnosis of arthritis and the diagnosis of uveitis, longer duration of uveitis, positive ANA, persistent oligoarthritis type of JIA, and use of corticosteroids or methotrexate before presentation were statistically significant risk factors for having at least one ocular complication at presentation.
|Table 1 Risk factors for presenting with at least one ocular complication|
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| Discussion|| |
JIA-associated uveitis is the most common cause of chronic uveitis in childhood and may result in significant visual impairment as a consequence of ocular complications such as cataract, band keratopathy, glaucoma, and macular pathology.
This study summarized the authors’ experience with JIA patients over 6 years, from 2010 to 2015. A referral bias exists because our institution is a tertiary-care medical center, and it is possible that only the most severe cases of JIA-associated uveitis were referred to us, as suggested by the high frequencies of ocular complications and the long duration of uveitis before presentation to our hospital.
There are also limitations inherent to the use of cross-sectional data, such as the difficulty in assessing temporal associations. Despite these limitations, this study suggests that structural ocular complications still occur frequently in JIA-related uveitis seen in the tertiary-care setting.
There appeared to be a significant delay in the referral of patients with JIA-related uveitis to our institution as the median duration of uveitis at the time of presentation to our institution was 6 years. In our cohort, a longer duration of uveitis at presentation was associated with the presence of at least one structural ocular complication and of poor vision at presentation in the univariate analyses .
We also observed that 44% of eyes affected with JIA-related uveitis had +1 anterior chamber cell at the time of presentation, and that this was associated with an almost three-fold increase in the odds of having an ocular complication (odds ratio, 2.88; P=0.005). Furthermore, because the leading causes of poor vision at presentation in this cohort were largely reversible causes of vision loss (cataract and band keratopathy) and because many children with JIA-related uveitis are at risk of developing amblyopia, prompt and aggressive control of intraocular inflammation in these patients to avoid sight-threatening complications is critical.
Although many clinical characteristics were observed to be significant risk factors for the presence of ocular complications at presentation in the univariate analyses, the presence of +1 anterior chamber flare appeared to be consistently associated with ocular complications at presentation after controlling for other variables.
We found that patients who were receiving methotrexate for their uveitis before or at the time of presentation had three-fold to four-fold higher odds of having an ocular complication at presentation than did patients with no history of methotrexate use. These findings, in conjunction with the increased risk of ocular complications among those who had received oral corticosteroids, likely represent a treatment bias by indication in which patients with more severe uveitis (who would be more likely to have ocular complications and visual impairment) are more likely to receive aggressive treatment than those with milder disease. The effect of immunosuppressive drugs on the incidence of visual impairment will require longitudinal data.
| Conclusion|| |
Poor vision and ocular complications still occur commonly among children with JIA-related uveitis, particularly if there is a long delay in referral to a tertiary-care center. Duration of uveitis, short duration between diagnosis of arthritis and uveitis, a positive ANA, and presence of at least +1 anterior chamber flare were associated with the presence of ocular complications at presentation. We recommend prompt referral of these patients to a uveitis specialist and early aggressive therapy with immunosuppressive agents, which may decrease the odds of poor vision.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Foster CS. Diagnosis and treatment of juvenile idiopathic arthritis-associated uveitis. Curr Opin Ophthalmol 2003; 14:395–398.
Kotaniemi K, Savolainen A, Karma A, Aho K. Recent advances in uveitis of juvenile idiopathic arthritis. Surv Ophthalmol 2003; 48:489–502.
Petty RE, Southwood TR, Manners P. International league of associations for rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390–392.
Berk AT, Koçak N, Ûnsal E. Uveitis in juvenile arthritis. Ocul Immunol Inflamm 2001; 9:243–251.
Wolf MD, Lichter PR, Ragsdale CG. Prognostic factors in the uveitis of juvenile rheumatoid arthritis. Ophthalmology 1987; 94:1242–1248.
Kanski JJ, Shun-Shin GA. Systemic uveitis syndromes in childhood: an analysis of 340 cases. Ophthalmology 1984; 91:1247–1252.
Ravelli A, Felici E, Magni-Manzoni S. Patients with antinuclear antibody-positive juvenile idiopathic arthritis constitute a homogeneous subgroup irrespective of the course of joint disease. Arthritis Rheum 2005; 52:826–832.
Chalom EC, Goldsmith DP, Koehler MA et al.
Prevalence and outcome of uveitis in a regional cohort of patients with juvenile rheumatoid arthritis. J Rheumatol 1997; 24:2031–2034.
Zulian F, Martini G, Falcini F. Early predictors of severe course of uveitis in oligoarticular juvenile idiopathic arthritis. J Rheumatol 2002; 29:2446–2453.
Chia A, Lee V, Graham EM, Edelsten C. Factors related to severe uveitis at diagnosis in children with juvenile idiopathic arthritis in a screening program. Am J Ophthalmol 2003; 135:757–762.
Key SN, Kimura SJ. Iridocyclitis associated with juvenile rheumatoid arthritis. Am J Ophthalmol 1975; 80:425–429.
Özdal PC, Vianna RN, Deschênes J. Visual outcomes of juvenile rheumatoid arthritis-associated uveitis in adults. Ocul Immunol Inflamm 2005; 13:33–38.
Dana MR, Merayo-Lloves J, Schaumberg DA, Foster CS. Visual outcomes prognosticators in juvenile rheumatoid arthritis-associated uveitis. Ophthalmology 1997; 104:236–244.
Edelsten C, Lee V, Bentley CR, Kanski JJ, Graham EM. An evaluation of baseline risk factors predicting severity in juvenile idiopathic arthritis associated uveitis and other chronic anterior uveitis in early childhood. Br J Ophthalmol 2002; 86:51–56.
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.
Lin DY, Wei LJ. The robust inference for the Cox proportional hazards model. J Am Stat Assoc 1989; 84:1074–1078.