Anti-adalimumab antibodies in juvenile idiopathic arthritis-related uveitis.

OBJECTIVES: To evaluate the association of adalimumab trough levels and anti-adalimumab antibodies with activity of uveitis in juvenile idiopathic arthritis-related uveitis. METHODS: This was a retrospective observational case series in a clinical setting at the Department of Ophthalmology, Helsinki University Hospital, Finland in 2014-2016. Thirty-one paediatric patients with chronic anterior juvenile idiopathic arthritis-related uveitis in 58 eyes and who had been on adalimumab ≥6 months were eligible for the study. Uveitis activity during adalimumab treatment, adalimumab trough levels and anti-adalimumab antibody levels were recorded. RESULTS: Anti-adalimumab antibody levels ≥12 AU /ml were detected in nine patients (29%). This level of anti-adalimumab antibodies was associated with a higher grade of uveitis (p<0.001), uveitis that was not in remission (p=0.001) and with lack of concomitant methotrexate therapy (p=0.043). In patients with anti-adalimumab antibody levels <12 AU/ml, higher serum trough levels did not associate with better control of uveitis (p=0.86). CONCLUSIONS: Adalimumab treatment might be better guided by monitoring anti-adalimumab antibody formation in treating JIA-related uveitis.

The frequency and outcome of uveitis in patients with newly diagnosed juvenile idiopathic arthritis in two 4-year cohorts from 1990-1993 and 2000-2003.

OBJECTIVES: To retrospectively compare the frequency and outcome of uveitis between two cohorts of patients with newly-onset juvenile idiopathic arthritis (JIA) separated by a 10 year interval. METHODS: The diagnosis of JIA was made in 239 patients in 1990-1993 and in 240 patients in 2000-2003 by paediatric rheumatologists at the Rheumatism Foundation Hospital, Heinola, Finland. An ophthalmologist examined all the patients regularly and diagnosed uveitis. The demographics of the patients, type of JIA, frequency, medical treatment and outcome of uveitis were documented. RESULTS: The main outcome measures were the frequency and outcome of uveitis, the number of complications and the best corrected visual acuity (BCVA), need of corticosteroids and other immunosuppressive treatment. The frequency of uveitis was higher (25% vs. 18%) in the earlier cohort. The visual outcome was ≥0.5 in all JIA-uveitis patients except one in the earlier cohort. Complications were fewer (21% vs. 35%) and uveitis was milder according to the Standardisation of Uveitis Nomenclature (SUN) criteria in the later cohort. Remission of uveitis (33% vs. 42%) and arthritis (20% vs. 23%) in JIA-uveitis patients was similar in both cohorts after a follow-up of 6.6 and 5.9 years, respectively. Systemic corticosteroids were more commonly used (25% vs. 7%) in JIA-uveitis patients of the earlier cohort but the use of methotrexate was equal in both cohorts (65% vs. 67%). CONCLUSIONS: In this study with early and aggressive treatment and close monitoring the outcome of JIA-uveitis patients was favourable and visual loss was avoided in most cases.

Diabetes, coeliac disease, multiple sclerosis and chronic arthritis in first-degree relatives of patients with juvenile idiopathic arthritis.

AIM: To evaluate the occurrence of autoimmune diseases in first-degree relatives of children with juvenile idiopathic arthritis (JIA) and to compare the figures with published population data. MATERIALS AND METHODS: Families of the 362 children with recently diagnosed JIA admitted to Rheumatism Foundation Hospital, Finland, from 1996 to 2001 were contacted by questionnaires regarding autoimmune diseases in family members. Data were collected on type 1 diabetes, coeliac disease, multiple sclerosis and chronic arthritis, consisting mainly of JIA, rheumatoid arthritis, spondyloarthropathy or psoriatic arthritis. RESULTS: In all, 21.4% of the 355 families with a patient with JIA had members with type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis. Thirty-three mothers and 23 fathers had type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis in 15.2% (95% CI 11.6-19.4) of the families, and 23 mothers and 15 fathers had chronic arthritis in 10.7% (95% CI 7.7-14.5) of the families. When compared with available research data, the prevalences of rheumatoid arthritis, spondyloarthropathy, psoriatic arthritis, paediatric type 1 diabetes and JIA (in siblings) were increased in JIA families. Coeliac disease was as prevalent as in the population. CONCLUSION: Autoimmune diseases cluster in families with a child with JIA.

Treatment of severe uveitis associated with juvenile idiopathic arthritis with anti-CD20 monoclonal antibody (rituximab).

OBJECTIVE: Rituximab (RTX), a chimeric mAb directed against the B-cell marker CD20, was investigated for its anti-inflammatory effect in treating refractory uveitis associated with JIA. METHODS: Case series, retrospective multicentre. JIA patients with severe uveitis with vision-threatening complications (n = 10) and with insidious onset. All patients were treated with RTX for active uveitis refractory to topical and systemic CSs, immunosuppressives and at least one of the TNF-α inhibitors. All had active arthritis. Uveitis and arthritis course were assessed before and after RTX treatment. RESULTS: After one RTX cycle (mean follow-up 11 months, range 7-18 months), uveitis inactivity was achieved in seven oligoarthritis patients (ANA⁺, HLA-B27⁻) for a prolonged period of time (mean 7.5 months, range 6-9 months). Therefore, CSs and immunosuppression could be spared. In three of four patients responding to RTX, uveitis recurred thereafter, and RTX re-treatment led to inactivity again. In another three patients (ANA⁺ polyarthritis, n = 1; ANA⁺ HLA-B27⁺ oligo- or polyarthritis, n = 2) uveitis activity persisted after RTX therapy. In seven patients, arthritis improved or was inactive after RTX treatment (PedACR30/50/70). CONCLUSION: RTX may represent a rescue therapy option for severe JIA-associated uveitis refractory to CSs, immunosuppression and TNF-α inhibitors.

Results 5 to 10 years after cataract surgery with primary IOL implantation in juvenile idiopathic arthritis-related uveitis.

PURPOSE: To evaluate the results of cataract extraction with primary intraocular lens (IOL) implantation in patients with juvenile idiopathic arthritis (JIA) and uveitis-related cataract. SETTING: Department of Ophthalmology, Helsinki University Hospital, Finland. DESIGN: Retrospective case series. METHODS: All consecutive patients younger than 20 years with JIA-uveitis-related cataract undergoing cataract extraction with primary IOL implantation in 1 or both eyes at the Department of Ophthalmology, Helsinki University Hospital, Finland, from February 2000 to April 2012 were included. Twenty eligible patients with 26 operated eyes were identified; 14 were girls and 6 were boys. All patients had a follow-up of 5 years and 13 patients (16 eyes [65%]) reached 10 years of follow-up. RESULTS: Twenty-six eyes of 20 patients were studied. Preoperative median corrected distance visual acuity (CDVA) was 0.05 in decimal notation. Median CDVA was 1.0 at 5 years and 0.9 at 10 years of follow-up. Two eyes did not reach CDVA 0.5 with the operation, and in 2 eyes, CDVA decreased below 0.5 over the period of 3 to 5 years after the operation. Active uveitis during 3 and 12 months preoperatively was a risk indicator for postoperative CDVA <0.5 at 5 years (P = .005 and P = .007, respectively). CONCLUSIONS: Cataract extraction with primary IOL implantation provides long-standing good visual acuity for young patients with well-controlled JIA-related uveitis.

Correction to: Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC).

Following publication of the original article [1], we have been notified that the author Joan Calzada should not have been included to the team of authors. The authors' team, thus, should be as follows.

Topical treatment of uveitis resulting in adrenal insufficiency.

UNLABELLED: The adverse effects of corticosteroids are well-known and occur more frequently when corticosteroids are used perorally or intravenously. The management of uveitis, which normally consists of topical corticosteroids and mydriates, can be challenging. We report a case in which continuous use of topical corticosteroids resulted in adrenal insufficiency and obesity. CONCLUSION: When topical corticosteroids are used over longer periods, hypophysis-pituitary-adrenal (HPA) function should be carefully monitored.

Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC).

BACKGROUND: JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinical outcome studies. METHODS: The group consists of 8 paediatric rheumatologists and 7 ophthalmologists. A consensus meeting took place on November 2015 in Barcelona (Spain) with the objective of validating the previously proposed measures. The validation process was based on the results of a prospective open, international, multi-centre, cohort study designed to validate the outcome measures proposed by the initial MIWGUC group meeting in 2012. The meeting used the same Delphi and nominal group technique as previously described in the first paper from the MIWGUC group (Arthritis Care Res 64:1365-72, 2012). Patients were included with a diagnosis of JIA, aged less than 18 years, and with active uveitis or an uveitis flare which required treatment with a disease-modifying anti-rheumatic drug. The proposed outcome measures for uveitis were collected by an ophthalmologist and for arthritis by a paediatric rheumatologist. Patient reported outcome measures were also measured. RESULTS: A total of 82 patients were enrolled into the validation cohort. Fifty four percent (n = 44) had persistent oligoarthritis followed by rheumatoid factor negative polyarthritis (n = 15, 18%). The mean uveitis disease duration was 3.3 years (SD 3.0). Bilateral eye involvement was reported in 65 (79.3%) patients. The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. We also found that visual analogue scales of disease activity showed significant change when reported by the ophthalmologist, rheumatologist and families. The measures formed three relatively distinct groups. The first group of measures comprised uveitis activity, ocular damage and the ophthalmologists' VAS. The second comprised patient reported outcomes including disruption to school attendance. The third group consisted of the rheumatologists' VAS and the joint score. CONCLUSIONS: We propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. This effort is the initial step for developing a comprehensive outcome measures for JIAU, which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.

Fluocinolone acetonide intravitreal implant (Retisert® ) in the treatment of sight threatening macular oedema of juvenile idiopathic arthritis-related uveitis.

PURPOSE: We describe eight patients with juvenile idiopathic arthritis-related chronic uveitis, who received a fluocinolone acetonide implant (FAI, Retisert®, Bausch&Lomb) in one eye. All patients had poor visual acuity (VA) due to persistent macular oedema in one or both eyes despite treatment with antirheumatic medication. METHODS: Median age of the patients was 22.9 years (range, 14.1-39.7) and duration of uveitis 13.0 years (range, 6.8-28.4) at FAI implantation. Median preoperative best-corrected visual acuity (BCVA) was 0.1 (range, 0.05-0.4) and Standardization of Uveitis Nomenclature, SUN-grade was SUN 2+ (range, 0.5-4.0). All patients had been treated extensively with systemic corticosteroids and antirheumatic drugs by the time of FAI implantation. The median follow-up time was 5.3 years (range, 4.4-6.3). RESULTS: Macular edema resolved in a median time of 0.2 years (range, 0.04-0.39) after the FAI implantation. The median BCVA was 0.5-0.63 (range, 0.1-1.0) from 1 to 5 years of follow-up. Macular edema did not recur in 5 eyes after the implantation. In three eyes, the macular oedema relapsed at 2.7, 2.9 and 5.5 years of follow-up. All our patients needed antirheumatic drugs in addition to the FAI to treat their macular edema. During the follow-up, 7 eyes required further intraocular operations: 4 cataract operations, 4 intraocular pressure -lowering operations and 1 retinal detachment surgery were performed. CONCLUSION: Fluocinolone acetonide implant is a valuable option in the treatment of persistent macular edema associated with JIA-related uveitis refractory to systemic treatments.

Evidence for Tocilizumab as a Treatment Option in Refractory Uveitis Associated with Juvenile Idiopathic Arthritis.

OBJECTIVE: To report on experience using the anti-interleukin 6 receptor antibody tocilizumab (TCZ) to treat severe and therapy-refractory uveitis associated with juvenile idiopathic arthritis (JIA). METHODS: Retrospective data were gathered from patients with JIA receiving TCZ treatment for uveitis. JIA and related uveitis data (disease onset, activity, structural complications, and topical and systemic antiinflammatory treatment) were evaluated at the start of TCZ (baseline) and every 3 months during TCZ therapy. RESULTS: A total of 17 patients (14 women) with active uveitis were included (mean age 15.3 ± 6.9 yrs, mean followup time 8.5 mos). In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, methotrexate (MTX), and other synthetic and biological disease-modifying antirheumatic drugs, including ≥ 1 tumor necrosis factor-α (TNF-α) inhibitor. Uveitis inactivity was achieved in 10 patients after a mean of 5.7 months of TCZ treatment (in 3 of them, it recurred during followup) and persisted in the remaining 7 patients. By using TCZ, systemic corticosteroids or immunosuppressives could be spared in 7 patients. Macular edema was present in 5 patients at baseline and improved in all of them under TCZ treatment. Arthritis was active in 11 patients at the initial and in 6 at the final followup visit. CONCLUSION: TCZ appears to represent a therapeutic option for severe JIA-associated uveitis that has been refractory to MTX and TNF-α inhibitors in selected patients. The present data indicate that inflammatory macular edema responds well to TCZ in patients with JIA-associated uveitis.

Potential Effect of Tumor Necrosis Factor Inhibitors on Trabeculectomy With Mitomycin C for Patients With Juvenile Idiopathic Arthritis-Related Uveitic Glaucoma: A Retrospective Analysis.

IMPORTANCE: The majority of patients with juvenile idiopathic arthritis-related uveitic glaucoma require surgery to control intraocular pressure. Trabeculectomy with mitomycin C (MMC) is a major treatment option, although both chronic inflammation and young age increase risk of filtration failure. Factors that potentially protect from filtration failure are important to identify. OBJECTIVE: To evaluate the potential effect of treatment with tumor necrosis factor (TNF) inhibitor on the success of an MMC-augmented trabeculectomy for patients with juvenile idiopathic arthritis-related uveitic glaucoma. DESIGN, SETTING, AND PARTICIPANTS: In a retrospective observational study with a median follow-up of 7.9 years at the Department of Ophthalmology, Helsinki University Hospital, in Helsinki, Finland, 29 eyes of 29 consecutive patients (3.1-20.4 years of age) underwent an MMC-augmented primary trabeculectomy during the period from April 1996 to January 2014. Fifteen patients were treated with systemic TNF inhibitors at the time of their trabeculectomy to control their uveitis, arthritis, or both. No changes were made to the antirheumatic therapy preoperatively. MAIN OUTCOMES AND MEASURES: Successful trabeculectomies, determined by Kaplan-Meier analysis, in which patients have intraocular pressure of 21 mm Hg or lower without antiglaucomatous medications or further glaucoma procedures. RESULTS: The success rate of trabeculectomy for patients who were treated with TNF inhibitors was 73% (95% CI, 44%-89%) at 1, 5, and 10 years after surgery, whereas the success rates of trabeculectomy for patients who were not treated with TNF inhibitors were 57%, 16%, and 0% at 1, 5, and 10 years after surgery, respectively (P = .01). The trabeculectomies of patients who were treated with TNF inhibitors were successful for a median of 3.2 years (95% CI, 0.3-9.9 years), whereas the trabeculectomies of patients who were not treated with TNF inhibitors were successful for a median of 1.2 years (95% CI, 0.6-3.6 years) (P = .14). Nine eyes of 9 patients had previously undergone cyclodestruction, intraocular surgery, or both (ie, prior ocular surgery). The effect of TNF was observed especially in the eyes of patients who had not undergone prior ocular surgery (at 5 years: 83% success rate for patients who had not undergone prior ocular surgery vs 19% success rate for patients who had). A difference in the overall success rate between patients who had and patients who had not had prior surgery was not identified. CONCLUSIONS AND RELEVANCE: Our retrospective data suggest that better control of intraocular pressure was achieved in the eyes of patients with juvenile idiopathic arthritis-related uveitis glaucoma who were treated with TNF inhibitors at the time of their MMC-augmented primary trabeculectomy. The retrospective design of the present study suggests that our data can be used for the planning of future studies but not for making treatment decisions.

Abatacept in the treatment of severe, longstanding, and refractory uveitis associated with juvenile idiopathic arthritis.

OBJECTIVE: Abatacept (ABA), a selective T cell costimulation modulator that binds to CD80 and CD86 on antigen-presenting cells, was investigated for its antiinflammatory effect in treating severe chronic uveitis associated with juvenile idiopathic arthritis (JIA). METHODS: Our retrospective study was conducted by members of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC). Patients with JIA who are receiving ABA treatment for active uveitis were included. In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, immunosuppressives, and at least 1 tumor necrosis factor-α inhibitor. A standardized protocol was used to document uveitis (MIWGUC) and arthritis. Baseline visit and visits at 3, 6, 9, and 12 months before and after ABA start were evaluated. Primary outcome measure was defined as achievement of uveitis inactivity; secondary outcome measures were tapering of corticosteroid and/or immunosuppressive treatment, and occurrence of complications. RESULTS: In all, 21 patients (16 female) with active uveitis (n = 21) and arthritis (n = 18) were included (mean age 11.8 ± 3.6 yrs). In 7 of 18 patients with active arthritis at baseline, inactivity was achieved following ABA treatment. Uveitis inactivity was achieved in 11 patients, but recurred later in 8 of them, and remained active in another 10 cases. Systemic corticosteroids or immunosuppression were tapered in 3 patients, but uveitis recurred in all of them during further followup. Ocular complications secondary to uveitis were present in 17 patients at baseline, while 3 patients developed new ocular complications during followup. CONCLUSION: A sustained response to ABA was uncommon in patients with severe and refractory uveitis.

Recent advances in uveitis of juvenile idiopathic arthritis.

Chronic scarring-type uveitis is a frequent extra-articular manifestation of juvenile idiopathic arthritis. It occurs in about 20% of children with this disease, commencing typically within a few years from its onset. The risk of uveitis is greatest in antinuclear antibody-positive girls with early onset oligoarthritis. The classic clinical picture is chronic bilateral anterior uveitis, usually asymptomatic until substantial damage to intraocular structures occurs. In view of the asymptomatic nature of the condition, routine screening of juvenile idiopathic arthritis patients 2-4 times a year is crucial to prevent complications. The treatment consists of topical corticosteroids and mydriatics, in severe cases with immunosuppressive agents, and surgical management of complications. Although the prognosis of uveitis is improving, there are cases refractory to standard regimens. Patients in whom uveitis commences prior to the onset of arthritis present a special problem.

Review for disease of the year: epidemiology of juvenile idiopathic arthritis and its associated uveitis: the probable risk factors.

Juvenile idiopathic arthritis (JIA) is the most common systemic disease associated with uveitis in childhood. The frequency of JIA-associated uveitis (JIAU) varies geographically, and between ethnicities. Uveitis risk is high in JIA associated with oligoarthritis, young age at arthritis onset and ANA positivity. Gender alters risk for the incidence of JIA and the severity of JIAU. Familial cases support the possible role of genetic influences in the pathogenesis. Arthritis typically precedes the uveitis, but uveitis may occur up to seven years following the arthritis onset. Although complications still occur, the frequency of bilateral blindness has dropped, probably by both improved screening of high-risk patients with JIA, and the increased use of early immunosuppression. However, there is still continuing persistence of JIAU into adulthood. For improvement of epidemiological knowledge of this complicated disease, large, well-defined, long-term population-based registries are needed with the application of universally agreed case definitions and outcome measures.

Proposed outcome measures for prospective clinical trials in juvenile idiopathic arthritis-associated uveitis: a consensus effort from the multinational interdisciplinary working group for uveitis in childhood.

OBJECTIVE: To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)-associated uveitis. METHODS: The literature relating to outcome measures used in studies of uveitis in childhood and adolescence was reviewed. A set of core outcomes and domains was established using the Delphi process. This was reviewed by a representative multinational interdisciplinary working group. Nominal group technique consensus was reached on face and content validity of the range and content of the domains. The outcomes and the appropriate instruments for uveitis trials were adapted to the age ranges of patients with JIA-associated uveitis. RESULTS: Consensus was reached that data should be reported at defined time points in longitudinal studies with patients stratified by prognostic markers. Visual acuity testing should be age appropriate. The severity of uveitis (measured as anterior chamber cell grade) and duration of active inflammation should be documented. Visually significant structural complications should be recorded and quantified with standard measures. The responses to treatment and corticosteroid-sparing effects of treatment should be documented. Patient-reported disease activity and age-specific uveitis-related quality of life should be reported using appropriate questionnaires. CONCLUSION: The proposed outcome measures in JIA-associated uveitis should aid in the standardization and comparison of future RCTs of the treatment regimens for this disease. The proposed outcome measures will be verified in a prospective validation study.

Long-term efficacy of adalimumab in the treatment of uveitis associated with juvenile idiopathic arthritis.

BACKGROUND: The purpose of this study was to investigate the long-term effects of adalimumab, a tumor necrosis factor alpha antagonist, in the treatment of uveitis associated with juvenile idiopathic arthritis. METHODS: Adalimumab was initiated in 94 patients with juvenile idiopathic arthritis to treat active arthritis and/or active associated uveitis. In 18 patients, therapy was discontinued after a short period because of inefficacy or side effects. The activity of uveitis (using Standardized Uveitis Nomenclature [SUN] criteria and clinical examination) and arthritis (number of swollen or active joints) was evaluated at the start and at end of the study. RESULTS: At the end of the study, uveitis was under good clinical control in two thirds of 54 patients (31% did not need any local treatment and 35% used only 1-2 corticosteroid drops a day), and one third had active uveitis (at least three corticosteroid drops a day). According to SUN criteria, adalimumab treatment for uveitis showed improved activity (a two-fold decrease in uveitis activity) in 28% of patients, with a moderate response in 16 patients, no change in a further 16 patients, and worsening activity (a two-fold increase in uveitis activity) in 13% of patients. The overall proportion of patients with active arthritis decreased. At the beginning of the study, 69% of patients with uveitis had more than two active joints, and at the end of the study only 27% had active joint disease. In 27 patients with juvenile idiopathic arthritis without uveitis on adalimumab, the number of active joints decreased from 93% to 59%. Systemic corticosteroid treatment could be stopped in 22% of patients with uveitis and in 11% of those without uveitis. Most of the patients had received methotrexate, other immunosuppressive therapy, or other biological drugs before initiating adalimumab. CONCLUSION: Adalimumab is a valuable option in the treatment of uveitis associated with active juvenile idiopathic arthritis.

An evaluation of dry eye symptoms and signs in a cohort of children with juvenile idiopathic arthritis.

OBJECTIVE: To determine the prevalence of dry eye symptoms and signs in children with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS: A total of 192 children with JIA: 48 oligo-, 39 extended oligo-, 79 polyarthritis, and 26 with other types of arthritis (eight juvenile spondyloarthritis, five juvenile psoriatic arthritis, three mixed connective tissue diseases, two systemic onset arthritis, and eight undetermined arthritis) were interviewed for dry eye symptoms and tested with Schirmer test with anesthetic. Two thirds of the patients were female and the mean age of the patients was 13.1 years (range 10-16) and the mean duration of arthritis was six years (SD 4, 4). Thirty-one percent of the patients had a history of uveitis. Dry eye was defined as Schirmer test score

Occurrence and management of ocular hypertension and secondary glaucoma in juvenile idiopathic arthritis-associated uveitis: An observational series of 104 patients.

AIMS: To describe the development and management of ocular hypertension (OHT) and secondary glaucoma (SG) in patients with juvenile idiopathic arthritis (JIA)-associated uveitis. PATIENTS AND METHODS: A series of 104 patients with newly diagnosed JIA and associated uveitis was collected in 1989-1996. A re-evaluation was made after mean 9.7 years (range 0.8-15.6 years) follow-up. OHT was diagnosed if intraocular pressure (IOP) had been >/=22 mmHg for longer than 3 months or when a single IOP was >/=30 mmHg despite normal visual field and optic disc. SG was diagnosed in a patient who had optic disc changes and/or visual field defects compatible with glaucoma. RESULTS: OHT or SG developed in 14 patients (14%, 22 eyes). IOP was under control (<22 mmHg) in 2 patients without treatment and in 3 patients with medication. Filtering surgery was performed in 9 patients, 5 of them needed additional glaucoma medication. The binocular visual acuity was 0.5 or better in all patients; in five eyes vision was less than 0.5, but no eye blinded. CONCLUSION: OHT or SG in JIA patients with uveitis is a diagnostic and therapeutic challenge, but if the medical and surgical treatment is timed correctly, the sight can be saved in most patients.