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Background: Currently, monoarticular Juvenile Idiopathic Arthritis (monoJIA) is included in the ILAR classification as oligoarticular subtype although various aspects, from clinical practice, suggest it as a separate entity., Objectives: To describe the clinical characteristics of persistent monoJIA., Methods: Patients with oligoJIA and with at least two years follow-up entered the study. Those with monoarticular onset and persistent monoarticular course were compared with those with oligoJIA. Variables considered were: sex, age at onset, presence of benign joint hypermobility (BJH), ANA, uveitis, therapy and outcome. Patients who had not undergone clinical follow-up for more than 12 months were contacted by structured telephone interview., Results: Of 347 patients with oligoJIA, 196 with monoarticular onset entered the study and 118 (60.2%), identified as persistent monoJIA, were compared with 229 oligoJIA. The mean follow-up was 11.4 years. The switch from monoarticular onset to oligoarticular course of 78 patients (38.8%) occurred by the first three years from onset. In comparison with oligoJIA, the most significant features of monoJIA were later age at onset (6.1 vs. 4.7 years), lower female prevalence (70.3 vs. 83.4%), higher frequency of BJH (61.9 vs. 46.3%), lower frequency of uveitis (14.4 vs. 34.1%) and ANA+ (68.6 vs. 89.5%) and better long-term outcome., Conclusions: MonoJIA, defined as persistent arthritis of unknown origin of a single joint for at least three years, seems to be a separate clinical entity from oligoJIA. This evidence may be taken into consideration for its possible inclusion into the new classification criteria for JIA and open new therapeutic perspectives., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objective: Anti-TNF-α agents have significantly changed the management of juvenile idiopathic arthritis (JIA). We evaluated the safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of JIA-associated uveitis in patients treated for ≥ 2 years., Methods: Patients with JIA-associated uveitis treated with IFX and ADA were managed by a standardized protocol and data were entered in the ORCHIDEA registry. At baseline, all patients were refractory to standard immunosuppressive treatment or were corticosteroid-dependent. Data recorded every 3 months were uveitis course, number/type of ocular flares and complications, drug-related adverse events (AE), and treatment switch or withdrawal. Data of patients treated for ≥ 2 years were analyzed by descriptive statistics., Results: Up to December 2014, 154 patients with ≥ 24 months followup were included in the study. Fifty-nine patients were treated with IFX and 95 with ADA. Clinical remission, defined as the absence of flares for > 6 months on treatment, was achieved in 69 patients (44.8%), with a better remission rate for ADA (60.0%) as compared to IFX (20.3%; p < 0.001). A significant reduction of flares was observed in all patients without difference between the 2 treatment modalities. The number of new ocular complications decreased in both groups but was lower for ADA (p = 0.015). No serious AE were recorded; 16.4% of patients experienced 35 minor AE and the incidence rate was lower with ADA than with IFX., Conclusion: At the 2-year followup, ADA showed a better efficacy and safety profile than IFX for the treatment of refractory JIA-associated uveitis.

Objective: Abatacept (ABA) has recently been proposed as second-line treatment in patients with juvenile idiopathic arthritis (JIA)-associated uveitis refractory to anti-tumor necrosis factor-α (anti-TNF) agents, but little is known about its efficacy as a first-line approach. The aim of the present study was to compare the safety and efficacy of ABA as a first-line biological agent (ABA-1) with that of ABA as a second-line treatment after 1 or more anti-TNF agents (ABA-2), in patients with severe JIA-related uveitis., Methods: In this multicenter study, we collected data on patients with severe JIA-related uveitis treated with ABA as a first-line or second-line biological agent. Changes in frequency of uveitis flares/year and ocular complications before and after ABA treatment, clinical remission, and side effects were recorded., Results: Thirty-five patients with a mean age of 10.8 years were treated with ABA for a mean period of 19.6 months. In 4 patients, ABA administration was discontinued, owing to inefficacy on arthritis in 3 cases and allergic reaction in 1. Thirty-one patients, 14 in the ABA-1 group and 17 in the ABA-2 group, completed the 12-month followup period; of these, 17 (54.8%) had clinical remission. The mean frequency of uveitis flares decreased from 4.1 to 1.2 in the ABA-1 group (p = 0.002) and from 3.7 to 1.2 in the ABA-2 group (p = 0.004). Preexisting ocular complications improved or remained stable in all but 5 patients, all in the ABA-2 group. No significant difference was found between the efficacy of the 2 treatment modalities. ABA confirmed its good safety profile., Conclusion: ABA, used as first-line biological treatment or after 1 or more anti-TNF agents, induces a comparable improvement in severe refractory JIA-related uveitis.

Objective: To evaluate the safety and efficacy of abatacept in patients with severe juvenile idiopathic arthritis (JIA)-related uveitis refractory or intolerant to immunosuppressive and anti-tumor necrosis factor alpha (anti-TNFalpha) agents., Methods: Patients with JIA-related uveitis refractory to immunosuppressive and anti-TNFalpha agents were treated with intravenous abatacept (10 mg/kg monthly). Side effects, frequency of uveitis flares, and ocular complications before and after treatment were reported., Results: Seven patients (6 females and 1 male) with a mean uveitis duration of 11.6 years entered the study. All patients had failed previous immunosuppressive therapy and >or=2 anti-TNFalpha treatments. All patients responded to abatacept and 6 maintained a clinical remission after a mean of 9.2 months of treatment. One patient withdrew from the study with oral mycosis and arthritis flare; no other patients had side effects. The mean frequency of uveitis flares during the 6 months before and after treatment decreased from 3.7 to 0.7 episodes. No new ocular complications or worsening of preexisting ones were reported., Conclusion: Abatacept treatment led to sustained improvement in severe anti-TNFalpha-resistant JIA-related uveitis and was well tolerated in all but 1 patient. These results provide new insights into a possible indication of abatacept for the treatment of uveitis.

Objective: To determine whether demographic, clinical, and laboratory variables at onset of arthritis can predict the development and the severity of anterior uveitis (AU) in oligoarticular juvenile idiopathic arthritis (JIA)., Methods: In a retrospective study, a cohort of 366 patients with oligoarticular onset JIA from 3 pediatric rheumatology centers were evaluated. Patients were classified in 3 groups: severe uveitis (SU) with a mean >/= 2 uveitis relapses/year with complications or need for immunosuppressive therapy; mild uveitis (MU) with a mean

To evaluate the long-term efficacy of methotrexate (MTX) monotherapy in patients with juvenile idiopathic arthritis-associated uveitis (JIA-U). We analyzed a cohort of patients with JIA-U treated with MTX monotherapy, divided into two groups depending on whether MTX was started before (on-MTX group) or after uveitis diagnosis (MTX-naïve group). The primary endpoint was the time between uveitis inactivity and first relapse. 84 patients entered the study. The median duration of remission on MTX monotherapy resulted 8.2 months. The on-MTX group showed a significant longer time interval between arthritis and uveitis onset and higher need for biologic agents (bDMARD). During follow-up, 40 patients (47.6%) needed bDMARD due to poor control of uveitis. Clinical remission off medication was achieved in 11.9% of patients, all belonging to the MTX-naïve group. MTX monotherapy, although effective in early stages of JIA-U, showed poor disease control in the long term. [ABSTRACT FROM AUTHOR]

Chronic anterior uveitis is the most important extra-articular complication of juvenile idiopathic arthritis. It is more frequent in the early-onset forms, with a higher prevalence in the oligoarticular subtype, and bilateral in most cases. The risk for visual impairment is still relevant due to sight-threatening complications, such as band keratopathy, cataract, glaucoma and cystoid macular edema. To date, treatment is not standardized and involves a complex decision-making process. Among several steroid-sparing immunosuppressive options, low-dose methotrexate is still the most diffuse treatment. Mycophenolate mofetil is another potential choice, although it is less effective in chronic anterior uveitis than in posterior or intermediate uveitis. TNF-α antagonists, the new generation of agents increasingly frequently used in autoimmune and rheumatic conditions, have demonstrated effectiveness in open-label studies, although no large, randomized, controlled trials have been reported so far. Although infliximab, an anti-TNF monoclonal antibody, seems to be superior to etanercept, an anti-TNF receptor antagonist, in controlling intra-ocular inflammation, serious side effects and loss of efficacy after the first year of treatment are reported. Adalimumab has evidenced efficacy similar to infliximab, but a better tolerance. In this review, the current practice in the medical management of juvenile idiopathic arthritis-related uveitis and the potential new agents are discussed. [ABSTRACT FROM AUTHOR]