JIA-associated Uveitis Research Articles

Granulomatous inflammation in juvenile idiopathic arthritis–associated uveitis

The uveitis that is associated with juvenile idiopathic arthritis (JIA) has typically been described as nongranulomatous. This study reports the prevalence of granulomatous disease in a population with JIA-associated uveitis. We conducted a retrospective analysis of all patients with JIA-associated uveitis seen between 1973 and 2006 at a hospital-based uveitis service. Patients with uveitis were included if they were diagnosed with JIA by a specialist and experienced at least 6 weeks of arthritis starting before the age of 16 years. Granulomatous disease was defined as Busacca or angle nodules, mutton-fat keratic precipitates (KP), or hyalinized "ghost" KPs. Statistical tests were performed to seek associations between granulomatous disease and various clinical factors. Seventy-one patients with JIA-associated uveitis were included. Granulomatous uveitis was observed in 28% of the population, with 7% exhibiting mutton-fat KP, and 21% "ghost" KP. There was no association between the presence of granulomatous disease and age of arthritis onset, age of uveitis onset, sex, antinuclear antibody status, or whether the arthritis was pauciarticular or polyarticular. In this population, 67% of black patients had granulomatous disease compared with 25% of nonblack patients (p < 0.05), and 24% of white patients showed granulomatous disease compared with 56% of nonwhite patients (p = 0.11). Granulomatous disease is more common in JIA-associated uveitis than previously thought and may be more common in black patients. Although it is necessary to exclude other forms of granulomatous uveitis, such as sarcoidosis, the presence of granulomatous uveitis is not inconsistent with a diagnosis of JIA-associated uveitis.

Risk factors and longterm outcome of juvenile idiopathic arthritis-associated uveitis in Switzerland.

To determine rate, risk factors, and longterm outcome of uveitis in children with juvenile idiopathic arthritis (JIA) in Switzerland and compare the results with a study of a different center in Switzerland from 1992. Retrospective analysis of the charts and ophthalmologists' reports of all patients with JIA in a tertiary care outpatient clinic between January 1, 1997, and December 31, 2005, for diagnosis, course, and outcome of uveitis. Uveitis occurred in 35/265 patients (13.2%) of our JIA cohort, which is similar to the 16% reported in the 1992 cohort. A positive test for antinuclear antibodies was the strongest risk factor. The JIA subgroup with the highest rate of uveitis was "other arthritis," followed by oligoarticular JIA. Extended and persistent course of oligoarticular JIA had a similar uveitis incidence, but all patients with extended-course disease developed uveitis before more than 4 joints were affected. After a mean followup of 5.62 years (range 0.5-15.17), 12/35 (34%) patients with uveitis had developed uveitis complications. Best corrected visual acuity was normal in 91% of patients. Only 5.6% of the affected eyes were legally blind as compared to 17.6% in the 1992 cohort. The rate of uveitis was 13.2% in our cohort of Swiss children and has not changed since 1992. Despite the high rate of uveitis complications, the longterm visual outcome was excellent.

Mycophenolate Mofetil after Methotrexate Failure or Intolerance in the Treatment of Scleritis and Uveitis

To evaluate the outcomes of treatment with mycophenolate mofetil in patients with scleritis and uveitis refractory to or intolerant of methotrexate. Retrospective noncomparative case series. Eighty-five patients with scleritis and/or uveitis who failed with or did not tolerate methotrexate and were subsequently treated with mycophenolate mofetil between 1998 and 2006. We reviewed medical records of patients who were treated with mycophenolate mofetil after methotrexate intolerance or failure at one tertiary uveitis referral practice. We recorded dose and duration of methotrexate and mycophenolate mofetil therapy, inflammation grade, Snellen visual acuity (VA), use of other immunomodulatory therapy, and adverse events. Multivariate logistic regression was used to identify factors associated with inflammation control. Control of inflammation, steroid-sparing effect, VA, and adverse effects were assessed. Inflammation was controlled with mycophenolate mofetil in 47 patients (55%), with 5 achieving durable remission off all medication. In multivariate logistic regression analysis that adjusted for gender and age, the odds of inflammation control were lower for patients with scleritis (odds ratio [OR], 0.19; 95% confidence interval [CI], 0.04-0.93; P = 0.04) than for patients without scleritis. Among patients without scleritis, the odds of inflammation control were lower for patients with juvenile idiopathic arthritis (JIA)-associated uveitis (OR, 0.14; CI, 0.02-0.81, P = 0.03) compared to patients without JIA-associated uveitis. Eight of the 11 patients (73%) who were taking concomitant prednisone were able to taper their dose to <10 mg daily. Visual acuity declined in a greater percentage of patients who were unresponsive to mycophenolate mofetil (29%) compared with that of patients who responded to mycophenolate mofetil (9%). Side effects requiring discontinuation of mycophenolate mofetil occurred in 18 patients (21%). Mycophenolate mofetil was effective in controlling inflammation in approximately half of the patients who had previously failed with or did not tolerate methotrexate. The odds of inflammation control were less in patients with the diagnoses of scleritis and JIA.

Cataract Surgery in Pediatric Uveitis

Uveitis in children is associated with several sight-threatening ocular complications, including the formation of cataracts. The surgical management of uveitic cataracts in children is both challenging and controversial and, unlike in adult uveitic cataracts, surgery has historically been associated with poor visual outcomes. Juvenile idiopathic arthritis-associated uveitis in particular poses unique therapeutic challenges and the issue of correction of aphakia in these patients remains a contentious one. The growing use of immunotherapies and, where needed, targeted biologic agents in childhood uveitis increases our potential to implant lenses and predict outcomes. The authors review the available evidence base for the treatment of these children.

Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis

To evaluate the efficacy of adalimumab in juvenile idiopathic arthritis (JIA)-associated uveitis. Retrospective observational study of 20 patients with JIA and chronic uveitis on adalimumab treatment. The ocular inflammation and improvement was assessed according to the Standardization of Uveitis Nomenclature criteria. At the initiation of adalimumab, the mean age of patients was 13.4 yrs and the mean duration of uveitis 8.7 yrs. Seventeen (85%) patients had polyarticular JIA and 19 (95%) had previously been on anti-TNF treatment. The mean duration of adalimumab therapy was 18.7 months. Of the 20 patients, 7 (35%) showed improved activity, 1 (5%) worsening activity and in 12 (60%) no change was observed in the activity of uveitis. Those with improved activity were younger and had shorter disease duration. The mean number of flares/yr decreased from 1.9 to 1.4 during adalimumab treatment. Serious adverse events or side-effects were not observed. Seven patients discontinued adalimumab during the follow-up: six because of inefficacy and one because of inactive uveitis. Adalimumab is a potential treatment option in JIA-associated uveitis, even in patients non-responsive to previous other anti-TNF therapy.

Was ist bei therapieresistenter rheumatischer Uveitis zu tun?

Die kindliche Uveitis tritt mit einer Inzidenz von 2,5 - 5/100 000 Personen auf und kann zahlreiche Ursachen haben. Die anteriore Uveitis ist am häufigsten mit einer Form der juvenilen idiopathischen Arthritis (JIA) assoziiert, meist einer Oligoarthritis, bei der sich antinukleäre Antikörper zu ca. 70 % nachweisen lassen, ist aber auch bei seronegativer Polyarthritis nicht selten. Der vorliegende Bericht versucht einen Überblick über die mit JIA assoziierten Uveitisformen zu geben, wobei der Hauptaspekt auf die Differenzierung der diversen Formen und die Therapie gelegt wird. Während die Prognose bei vielen der als schwer eingestuften ca. 25 % aller JIA-assoziierten Uveitis Kinder früher schlecht war, was u. a. auf dem relativ späten Einsatz von Immunsuppressiva (IS) beruhte, werden IS in den letzten Jahren schneller verordnet, mit Verbesserung der Prognose durch Reduktion der Komplikationen. Es scheint, dass die früher als therapierefraktär eingestuften Kinder, die nicht ausreichend auf IS angesprochen haben, nun sehr häufig gut auf TNF-alpha-Blocker, besonders Infliximab und Adalimumab, reagieren. Zusätzlich wird eine der Grunderkrankung adäquate Empfehlung für die Kontrollintervalle gegeben.

Risk Factors for the Development of Cataract Requiring Surgery in Uveitis Associated with Juvenile Idiopathic Arthritis

To identify the possible risk factors for the development of cataract requiring surgery in children with juvenile idiopathic arthritis (JIA)-associated uveitis. Retrospective cohort study. Data of 53 children with JIA-associated uveitis, of whom 27 had undergone cataract extraction (CE), were obtained. The main outcome measure, the interval between the onset of uveitis and the first CE (U-CE interval), was examined in relation to clinical and ophthalmologic characteristics and treatment strategies before CE. A shorter U-CE interval was found for children with posterior synechia vs those without posterior synechia (hazard ratio [HR], 3.57; 95% confidence interval [CI], 1.33 to 10.00). No significant difference was found for children in whom the uveitis was the first manifestation of JIA vs those in whom arthritis was the first manifestation of JIA (HR, 1.59; 95% CI, 0.63 to 4.00) and children treated with periocular corticosteroid injections vs those not treated with periocular corticosteroid injections (HR, 3.23; 95% CI, 0.95 to 11.11). Children treated with methotrexate (MTX) had a longer U-CE interval than children not treated with MTX (HR, 0.29; 95% CI, 0.10 to 0.87). The risk factor for development of early cataract requiring surgery in children with JIA-associated uveitis is the presence of posterior synechia at the time of diagnosis of uveitis. However, early treatment with MTX is associated with a mean delay in the development of cataract requiring surgery of 3.5 years.

Resolution of Juvenile Idiopathic Arthritis-Associated Uveitis after Development of Common Variable Immunodeficiency

To describe the occurrence of common variable immunodeficiency (CVID) in a patient with juvenile idiopathic arthritis (JIA) and JIA-associated uveitis. Case report. A 29-year-old woman was followed-up since the age of 10 years because of right eye JIA-associated recurrent anterior uveitis. She was treated with steroids and immunosuppressants with good control of uveitis and arthritis. At the age of 17 years, she did not experience any further relapse of uveitis or arthritis and both diseases were considered to be in remission. Concomitantly, she started to have recurrent infections and later she underwent splenectomy because of autoimmune hemolytic anemia and thrombocytopenia. Liver biopsy disclosed granulomatous hepatitis. She was ultimately diagnosed with CVID at the age of 23 years when her blood tests revealed neutropenia and severe panhypogammaglobulinemia. She has been treated since then with intravenous immunoglobulins with good control of the disease. Since the development of CVID, she has had no relapses of uveitis or arthritis during a follow-up period of 12 years. Common variable immunodeficiency (CVID) is the most common primary immunodeficiency where defective antibody formation is the most common feature with B-cell differentiation failure. Ocular complications have been rarely documented and included bacterial conjunctivitis, retinal vasculitis and multifocal choroiditis. We herein report on the occurrence of JIA-associated uveitis as a comorbid manifestation of CVID. We speculate a role for B cells in the pathogenesis of JIA and JIA-associated uveitis here, as this patient had total remission of both conditions with the onset of CVID.

EBM Analyse: Klassische DMARDs (Disease modifying antirheumatic drugs) und Immunsuppressiva bei Arthritis und Uveitis

The best experience in the use of DMARDs/immunosuppressive drugs for treatment of juvenile idiopathic arthritis (JIA) and JIA-associated uveitis has been obtained with MTX. Controlled studies on the treatment of uveitis in JIA, however, are still lacking; this is also true for other DMARDs/immunosuppressive drugs. Thus, the grading of the evidence level for these substances only reaches evidence level III (expert opinion, clinical experience or descriptive studies). For the treatment of uveitis in JIA the utility of MTX in comparison to the new biological substance classes (e. g., tumor necrosis factor-alpha, blockers, interleukin-1 receptor antagonist) will have to be examined. Experience with Mycophenolate mofetil (MMF) in JIA uveitis is limited to single cases. According to data from a retrospective analysis cyclosporin A (CsA) appears to have a limited efficacy in JIA uveitis. The results with azathioprine are not consistent. Controlled studies which led to the approval of drugs for JIA are also mandatory for uveitis to offer the most effective and safe therapy for children.

Biologika zur Therapie der juvenilen idiopathischen Arthritis - Update 2007

Mit der Erstzulassung des TNF-α-Antagonisten Etanercept zur Behandlung der juvenilen polyartikulären Arthritis vor nunmehr 8 Jahren wurden die Behandlungsmöglichkeiten revolutionär erweitert. Die heute zur Verfügung stehenden drei TNF-Antagonisten Etanercept, Adalimumab und Infliximab erscheinen gleichwertig effektiv bei der Behandlung der Arthritis, wenngleich keine Studien zum Direktvergleich zur Verfügung stehen. Die profunde Entzündungshemmung bewirkt eine Verminderung subjektiver Beschwerden, Morgensteifigkeit, Gelenkschmerzen, Müdigkeit/Fatigue, Verhinderung von Knorpel- und Knochendestruktionen und Aufholwachstum bei der weit überwiegenden Mehrheit der Patienten bei überraschend guter Verträglichkeit. Extraartikuläre Manifestationen, wie z. B. die chronische Uveitis und die systemischen Manifestationen im Rahmen eines Still-Syndroms lassen sich nicht mit gleicher Zuverlässigkeit kontrollieren. Adalimumab scheint aber für den Einsatz bei der JIA-assoziierten Uveitis besonders geeignet. Aufgrund der Verschiedenheit der Zytokindysregulation beim Still-Syndrom ist die Blockade der proinflammatorischen Zytokine Interleukin-1β und Interleukin-6 mit dem Interleukin-1-Rezeptoranatonisten Anakinra oder dem Interleukin-1-Rezeptor Fusionsprotein Rilonacept bzw. mit Tocilizumab, einem Antikörper gegen den Interleukin-6-Rezeptor, bereits in klinischen Studien erfolgreich untersucht. Neben der Zytokinblockade ist die Ausschaltung der Aktivierung von T-Zellen durch Blockade kostimulatorischer Moleküle mit Abatacept, einem CTLA4-IgG-Fusionsprotein, erfolgreich. Die vorliegende Übersicht soll den aktuellen Stand dieser neuen Therapiestrategien der differenziellen Zytokinblockade und der Aktivierungsblockade bei der Therapie der juvenilen idiopathischen Arthritis darstellen.

Juvenile Idiopathic Arthritis-Associated Uveitis: Incidence of Ocular Complications and Visual Acuity Loss

To estimate the incidences of ocular complications and vision loss in patients with juvenile idiopathic arthritis (JIA)-associated uveitis, to describe risk factors for vision loss, and to describe the association between therapy and complications and vision loss. Retrospective cohort study. setting: Single-center, academic practice. study population: A total of 75 patients with JIA-associated uveitis evaluated between July 1984 and August 2005. procedures: Clinical data on these patients were analyzed. outcome measures: Occurrence of ocular complications and visions of 20/50 or worse and 20/200 or worse. Over a median follow-up of three years, the incidence of any ocular complication was 0.33/eye-year (EY). Rates of vision loss to 20/50 or worse and 20/200 or worse were 0.10/EY and 0.08/EY, respectively. Risk factors at presentation for incident vision loss included presence of posterior synechiae, anterior chamber flare > or = 1+, and abnormal intraocular pressure (IOP). During follow-up, ocular inflammation > or = 0.5+ cells was associated with an increased risk of visual impairment (relative risk [RR] = 2.02, P = .006) and of blindness (RR = 2.99, P = .03). Immunosuppressive drug therapy reduced the risk of hypotony by 74% (P = .002), epiretinal membrane formation by 86% (P = .05), and blindness in the better eye by 60% (P = .04). Incident vision loss and complications were common. Presence of posterior synechiae, anterior chamber flare > or = 1+, and abnormal IOP at presentation were associated with vision loss during follow-up. Use of immunosuppressive drugs reduced the risk of some ocular complications and of blindness in the better-seeing eye.

Prevalence, risk factors, and outcome of uveitis in juvenile idiopathic arthritis: A long‐term followup study

To assess the prevalence, risk factors, and long-term outcome of uveitis in patients with juvenile idiopathic arthritis (JIA). An inception cohort of all 1,081 patients diagnosed as having JIA at a single tertiary care center was established. A questionnaire and followup telephone calls were used to confirm the diagnosis of uveitis. Ophthalmologists' records of patients with uveitis were collected. Kaplan-Meier and Cox regression analyses were used to assess risk factors for developing uveitis and for complications of uveitis. After a mean followup time of 6.9 years, 142 of 1,081 patients (13.1%) had developed uveitis. Risk factors were young age at diagnosis, female sex, antinuclear antibody positivity, and the subtype of JIA. The relative contribution of these risk factors was different for the different subtypes of JIA. Until the end of the study, uveitis complications had developed in 53 of 142 patients with uveitis (37.3%; 4.9% of the total cohort). Only 16 of 175 involved eyes (9.1%) in 14 of 108 patients (13%; 1.3% of the total cohort) for whom ophthalmology reports were available had best corrected visual acuity less than 20/40 (mean followup time for uveitis of 6.3 years). Abnormal vision was associated with synechiae or cataract. Risk factors for developing uveitis were different among subtypes of JIA. The long-term outcome of JIA-associated uveitis in our cohort was excellent despite the high rate of complications.

Surgery for Hypotony in Patients with Juvenile Idiopathic Arthritis-Associated Uveitis

Purpose: To describe the clinical response to cyclitic membrane excision of eyes with juvenile idiopathic arthritis (JIA)-associated uveitis and hypotony. Methods: The clinical records of patients with JIA-associated uveitis in a tertiary referral center were reviewed. Those patients with JIA-associated uveitis and hypotony who underwent cyclitic membrane excision were included in the study. Patients with hypotony secondary to active inflammation, retinal detachment, and surgical intervention (filtering, cyclo-destructive procedures, and glaucoma valve implantation) were excluded. Results: Two pediatric and two adult patients (4 eyes) were identified. The range of pre-operative intraocular pressure (IOP) was 0–5 mmHg. The two adult patients were noted to have atrophic ciliary processes intra-operatively, while the two pediatric patients had normal ciliary processes. At six months follow-up, the adult patients had IOPs of 5 mmHg, while the two pediatric patients had IOPs of 16 mmHg. At last consultation (mean duration of follow-up: 3.6 years), IOP was normal in all eyes. None of the eyes had a decrease in vision. Conclusions: Release of traction on the ciliary body by inflammatory membranes may play a role in the management of hypotony in patients with JIA-associated uveitis. However, even though the IOP was successfully elevated, preventing phthisis, vision remained poor due to the long-standing complications secondary to chronic uveitis. This emphasizes the critical importance of early diagnosis and appropriate treatment of the inflammation before vision-robbing complications occur.

Risk Factors for Ocular Complications and Poor Visual Acuity at Presentation Among Patients With Uveitis Associated With Juvenile Idiopathic Arthritis

To describe the frequencies of and risk factors for ocular complications and poor visual acuity at presentation in a cohort of patients with juvenile idiopathic arthritis (JIA)-associated uveitis. Cross-sectional study. setting: Single-center, academic practice. study population: Seventy-five patients with JIA-associated uveitis were evaluated between July 1984 and August 2005. observation procedures: Data on patients diagnosed with JIA-associated uveitis were entered retrospectively into a database and analyzed. outcome measures: Visual acuity of 20/50 or worse or 20/200 or worse, and presence of ocular complications (including cataract, posterior synechiae, band keratopathy, elevated intraocular pressure, hypotony, macular edema, and epiretinal membrane) at presentation. At presentation, ocular complications were seen in 67% of eyes affected by JIA-associated uveitis. Presence of > or =1+ anterior chamber flare, a positive antinuclear antibody (ANA), and a shorter duration between the diagnosis of arthritis and uveitis were significantly associated with the presence of ocular complication. The frequencies of 20/50 or worse and of 20/200 or worse visual acuities at presentation in affected eyes were 36% and 24%, respectively. The presence of > or =1+ anterior chamber flare and a history of intraocular surgery before presentation were significantly associated with 20/50 or worse and 20/200 or worse vision. Presence of posterior synechiae also was associated with 20/200 or worse vision at presentation. The main causes of poor vision at presentation for affected eyes and better-seeing eyes were cataract, band keratopathy within the visual axis, and glaucoma. Ocular complications and poor vision at presentation were common in our patients with JIA-related uveitis.

Update on the medical treatment of juvenile idiopathic arthritis

Many exciting developments in the treatment of juvenile idiopathic arthritis (JIA) have emerged recently, including new tools to assess the results of clinical trials (eg, the definition of remission and a radiologic scoring tool). New controlled studies examined the equivalence of meloxicam to naproxen, the efficacy of leflunomide but the superiority of methotrexate, and the use of infliximab in polyarthritis JIA. Initial studies have shown the potential of anti-interleukin (IL)-1 and anti-IL-6 receptor antibody therapy for systemic JIA. Corticosteroid-sparing medications including the use of "biologic modifiers" for JIA-associated uveitis have been described. Evidence-based guidelines for the main subtypes of JIA have been published. However, good evidence on the treatment of several disease subtypes is still lacking. Studies of new medications and the use of combination therapy, including aggressive induction therapy early in the disease course, are necessary to continue improving the outcome of JIA patients.

Visual Outcomes in Children with Juvenile Idiopathic Arthritis–Associated Uveitis

To analyze visual outcomes in children affected by juvenile idiopathic arthritis (JIA)-associated uveitis. Retrospective interventional case series. Eighty-nine children with JIA-associated uveitis. Charts of children with JIA-associated uveitis were reviewed. Change in patients' visual acuities (VAs). Of 269 children with uveitic syndromes referred, 89 (33%) had JIA-associated uveitis. The process was bilateral in 76 children. Seventy-three patients were female, and 84% of patients were Caucasian. Mean age of onset of uveitis was 5.7 years. Mean follow-up was 2.96 years. Antinuclear antibody positivity was detected in 56 patients, 44 of them female. Patients with JIA-associated uveitis developed numerous complications in the course of their disease: of 165 affected eyes, 105 (64%) developed cataracts, 33 (20%) developed increased intraocular pressure, and 76 (46%) developed band keratopathy; posterior synechiae were present in 96 (58%). Of 89 children, 73% were treated with immunomodulators, 40% were treated with nonsteroidal antiinflammatory agents alone or in combination with immunomodulators, and 21% were treated with topical and/or systemic steroids. Of 65 children who required immunomodulation, only one chemotherapeutic agent was used in 30, two agents in 21, and > or =3 in 14. Visual acuities of 65 children (122 eyes) were documented and compared at standard intervals. By mixed-models linear regression, improvement in VA of 0.03 logarithm of the minimum angle of resolution units per year was not found to be statistically significant (standard error, 0.02, P = 0.089). Juvenile idiopathic arthritis-associated uveitis is a sight-threatening disease. However, much of the children's vision can be preserved if patients are treated appropriately.

Ocular Hypertension and Secondary Glaucoma in Children with Uveitis

To identify the risk factors for ocular hypertension and secondary glaucoma in children with uveitis. Retrospective observational case series of 147 patient records. Two hundred fifty-six eyes of 147 children with uveitis diagnosed before the age of 16 years. Data were obtained from the medical records of children with uveitis evaluated at our institute from 1990 through 2004. Localization and course of uveitis (acute or chronic), underlying systemic disease, onset of ocular hypertension, onset of secondary glaucoma, treatment with steroids, antinuclear antibodies (ANAs), lens extractions, number of blind eyes at onset and during follow-up, and the duration of follow-up. Elevated intraocular pressure developed in 35% of children with pediatric uveitis regardless of the form or type of uveitis during a follow-up of 5 years. Secondary glaucoma, however, developed more frequently in juvenile idiopathic arthritis-associated uveitis (38%) compared with other forms of uveitis (11%) and more frequently in children with uveitis who were ANA positive (42%) than in those who were ANA negative (6%). Elevated intraocular pressure occurred in two thirds of all children within the first 2 years after the diagnosis of uveitis. Except for patients with juvenile idiopathic arthritis-associated uveitis, periocular steroid injections represented an additional risk factor for secondary glaucoma, but this risk was limited to the early phase of the disease process. In children with uveitis in this series, juvenile idiopathic arthritis-associated uveitis and ANA-positive uveitis without evidence of arthritis are the most important risk factors for developing secondary glaucoma.

Extended Oligoarthritis and Other Risk Factors for Developing JIA-Associated Uveitis Under ILAR Classification and Its Implication for Current Screening Guideline

Purpose: To investigate the risk factors for developing uveitis in a regional cohort of patients with juvenile idiopathic arthritis (JIA) as classified under ILAR criteria. Patients and Methods: The clinical factors for developing uveitis and its visual outcome were studied retrospectively for all children diagnosed with JIA at Nottingham University Hospital, England from 1974 to 2001. Results: A total of 202 patients with juvenile idiopathic arthritis were identified. Twenty-three patients (11.4%) were found to have uveitis. The mean age of arthritis onset in those with uveitis was 4.9 (95% CI 3.4–6.4) and in those without uveitis was 7.6 (95% CI 7.0–8.3), p = 0.002. Both the persistent and extended oligoarthritis groups are at significant risk of developing uveitis on Kaplan-Meier analysis with p = 0.001 and 0.013, respectively, compared to other ILAR subtypes. Extended oligoarthritis (1 to 4 joints affected in first 6 months of disease but 5 or more cumulative joints after first 6 months) had the highest prevalence of uveitis (25%) among the ILAR subtypes. Patients with extended oligoarthritis also developed uveitis earlier than persistent group, p = 0.017. Gender, race, and antinuclear antibody (ANA) status were not significant risk factors. The visual outcome was favorable, with 90% achieving acuity of 6/12 or better. Conclusion: Patients with extended oligoarthritis are at higher risk and have a shorter interval from diagnosis of arthritis to development of uveitis and need to be monitored more closely. Screening guideline for JIA-associated uveitis based on ILAR classification is called for.

Infliximab for juvenile idiopathic arthritis‐associated uveitis

Infliximab is a murine-human recombinant antitumour necrosis factor monoclonal antibody recently introduced for the treatment of autoimmune diseases in which tumour necrosis factor is thought to be a key mediator. Its role in the treatment of juvenile idiopathic arthritis-associated uveitis is as yet undefined. Six children with juvenile idiopathic arthritis-associated uveitis, inadequately controlled on currently available therapy, were treated with infliximab between September 2002 and November 2004. All children were required to remain on low-dose immunomodulatory treatment in conjunction with the infliximab. A retrospective review of two electronic databases containing details of ophthalmology and rheumatology visits was conducted. In all six children, institution of infliximab therapy was associated with increased ease of management. Ocular inflammation and intraocular pressure control improved in all. It was also possible to reduce the dose or withdraw some glaucoma, steroid and other immunomodulatory drugs. Two children underwent intraocular surgery without noticeable flare of intraocular inflammation. No patient developed any serious systemic complications attributable to infliximab. Infliximab may be a useful adjunct to the management of refractory juvenile idiopathic arthritis-associated uveitis. In our series it was associated with improved uveitis control and simplification of drug use as well as possibly improving safety of surgical intervention. This study suggests that its role is likely to be in conjunction with maintenance immunomodulatory treatment to provide more optimal disease control. Controlled studies are required to confirm its efficacy and safety, and the potential breadth of its use in uveitis and related disorders.

Outcomes of Treatment with Immunomodulatory Therapy in Patients with Corticosteroid-Resistant Juvenile Idiopathic Arthritis-Associated Chronic Iridocyclitis

Purpose: To describe the clinical outcome of patients with juvenile idiopathic arthritis (JIA)-associated chronic iridocyclitis unresponsive to conventional therapy, in whom inflammation was eventually controlled using immunomodulatory therapy (IMT), and to determine if patients treated early with IMT have better visual acuity outcomes than those treated with corticosteroid alone. Methods: Patients with JIA-associated chronic iridocyclitis receiving immunomodulatory therapy at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary between 1981 and 2001 were studied. Inclusion criteria: JIA with chronic corticosteroid-dependent uveitis; IMT for a minimum of one year; minimum follow-up of three years; inflammation eventually controlled for a minimum of six consecutive months by IMT. Results: At last visit, 51% of eyes (23 eyes) had acuity of 20/20 to 20/40, 16% (7 eyes) had 20/50 to 20/100, and 33% (15 eyes) had 20/200 or less. In patients whose chronic inflammation was eventually controlled within three years from onset of the uveitis, final bilateral vision was maintained within 20/20 to 20/30, except for a patient whose one eye had poor vision at initial consultation. When acuities at the endof the follow-up period of patients treated early with IMT were compared with acuities at the initial consultation of patients treated late with IMT, with duration of uveitis matched for each patient, visual acuities of those treated early were statistically significantly better than those treated late with IMT (p < 0.005 right and left eyes pooled; p = 0.0075 best eyes; p = 0.0375 worst eyes). Conclusions: We noted improvement or maintenance of visual acuity (86%) during the course of follow-up of patients with treatment-resistant JIA-associated uveitis treated with effective IMT. However, only IMT given early in the disease course was noted to be associated with bilateral visual acuity of 20/30 or better.