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Abstract Background Juvenile idiopathic arthritis (JIA) refers to a heterogeneous group of rheumatic conditions in children. Novel drugs have greatly improved disease outcomes; however, outcomes are impacted by limited awareness of the importance of early diagnosis and adequate treatment, and by differences in access across health systems. As a result, patients with JIA continue to be at risk for short- and long-term morbidity, as well as impacts on virtually all aspects of life of the child and family. Main body Literature on the socioeconomic burden of JIA is largely focused on healthcare costs, and the impact of JIA on patients, families, and communities is not well understood. High quality evidence on the impact of JIA is needed to ensure that patients are receiving necessary support, timely diagnostics, and adequate treatment, and to inform decision making and resource allocation. This commentary introduces the European Joint Programme on Rare Diseases: Producing an Arthritis Value Framework with Economic Evidence: Paving the Way for Rare Childhood Diseases (PAVE) project, which will co-develop a patient-informed value framework to measure the impact of JIA on individuals and on society. With a patient-centered approach, fundamental to PAVE is the involvement of three patient advocacy organizations from Canada, Israel, and Europe, as active research partners co-designing all project phases and ensuring robust patient and family engagement. The framework will build on the findings of projects from six countries: Canada, Germany, Switzerland, Spain, Israel, and Belgium, exploring costs, outcomes (health, well-being), and unmet needs (uveitis, mental health, equity). Conclusion This unique international collaboration will combine evidence on costs (from family to societal), outcomes (clinical, patient and family outcomes), and unmet needs, to co-design and build a framework with patients and families to capture the full impact of JIA. The framework will support the development of high-quality evidence, encompassing economic and clinical considerations, unmet needs, and patient perspectives, to inform equitable resource allocation, health system planning, and quality of care better aligned with the needs of children with JIA, their families, and communities. Knowledge gained from this novel approach may pave the way forward to be applied more broadly to other rare childhood diseases.

Abstract Background Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). Methods Observational patient cohort study with 201 recruited children from 4 countries (3 European, 1 North Africa) from 2005 until 2019, using retrospectively (2005–2015) and prospectively (2015–2019) routine care collected data. Results Sixty-five patients with complete follow-up data for 24 months after first diagnosis were classified as monophasic (n = 23), polyphasic (n = 6) or persistent group (n = 36) corresponding to their evolution (unique flare, recurrent flares, or persistent disease activity respectively). The patients of the persistent group were more likely to have an earlier disease onset, before the age of 6 (OR 2.57, 95%-CI 0.70–9.46), persistence of arthritis at 12-months post-diagnosis (OR 4.45, 95%-CI 0.58–34.20) and higher use of synthetic DMARD (sDMARD, OR 5.28, 95%-CI 1.39–20.01). Other variables like global assessment by physician and by patient and C Reactive Protein levels at 12-months post-diagnosis were assessed but without any predictive value after adjusting for confounding factors. Conclusions Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course.

Abstract Familial Mediterranean Fever (FMF) is the most common inherited auto inflammatory disease, characterized by recurrent, self–limited attacks of fever and aseptic polyserositis.FMF is widespread in Armenia and there is a higher than expected frequency of FMF-associated arthritis in children. Chronic arthritis can be the first, and sometimes the only manifestation of FMF.

Abstract Background We evaluated the effect of infliximab on markers of inflammation in patients with juvenile idiopathic arthritis (JIA). Methods In this randomized, placebo-controlled substudy, 122 patients with JIA received infliximab 3 mg/kg + methotrexate (MTX)(n = 60) or placebo + MTX (n = 62) at weeks 0, 2, and 6. At week 14, patients receiving placebo + MTX crossed over to infliximab 6 mg/kg + MTX; patients receiving infliximab 3 mg/kg + MTX continued treatment through week 44. Sera and plasma from eligible patients receiving infliximab 3 mg/kg + MTX (n = 34) and receiving placebo→infliximab 6 mg/kg +MTX (n = 38) were collected at weeks 0, 2, 14, 16, 28, and 52 and analyzed for inflammatory markers (IL-6, IL-12p40, ICAM-1, MMP-3, VEGF, TNF-α, and CRP). Results At week 2, decreases from baseline in IL-6, ICAM-1, MMP-3, TNF-α, and CRP were greater with infliximab versus placebo treatment, and with the exception of CRP, these differences were generally maintained through week 14. The decreases from baseline to week 52 in IL-6, ICAM-1, VEGF, MMP-3, and CRP and increases in IL-12p40 levels were larger in patients receiving placebo→infliximab 6 mg/kg +MTX versus infliximab 3 mg/kg + MTX treatment. Patients receiving infliximab 3 mg/kg+MTX who achieved an American College of Rheumatology Pediatric 30 (ACR-Pedi-30) response had significantly larger decreases from baseline in ICAM-1 (p = 0.0105) and MMP-3 (p = 0.0253) at week 2 and in ICAM-1 (p = 0.0304), MMP-3 (p = 0.0091), and CRP (p = 0.0011) at week 14 versus ACR-Pedi-30 nonresponders. Conclusion Infliximab + MTX attenuated several inflammatory markers in patients with JIA; larger decreases in ICAM-1, MMP-3, and CRP levels were observed in ACR-Pedi-30 responders versus nonresponders. Trial Registration NCT00036374

Juvenile idiopathic arthritis (JIA) is a leading cause of acquired disability and malformation in childhood. Although virtually any joint can be affected, one of the most commonly involved yet least frequently recognized joints is the temporomandibular joint (TMJ). 1 Persistent arthritis can result in pain, growth problems, cosmetic defects, and poor function, underscoring the necessity of obtaining early control of TMJ arthritis. While the therapy of JIA as a whole has benefitted enormously from the introduction of novel therapeutic agents, 2 it remains uncertain to what extent these therapies benefit the TMJ specifically. Thus, many practitioners use intra-articular therapy for refractory TMJ arthritis, although concerns about their long-term safety have been raised. 3 Herein, we will review the data on systemic and local anti-inflammatory therapy of TMJ arthritis. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical features of uveitis in patients with juvenile idiopathic arthritis (JIA). Methods: Retrospective chart review of a subset of 1,081 consecutive JIA patients who were younger than 18 years of age and had uveitis, with a minimum of 1-year follow-up at a single center. Results: One hundred forty-two patients (13.1%) developed uveitis after a mean follow-up of 6.3 years (range, 0.10-23.2). Uveitis types were chronic anterior (97/142, 68.3%), acute anterior (23/142, 16.2%), recurrent anterior (17/142, 12%), and panuveitis (5/142, 3.5%). Uveitic complications were observed in 37.3% of cases (53/142) and 32.5% of eyes (74/228). When we compared uveitic eyes with complications to uveitic eyes without complications, we found the following significant differences: time interval from diagnosis of JIA to diagnosis of uveitis was shorter (mean, 1.3 years vs. 2.2 years; p = 0.003) and use of oral prednisone was greater (59.1% vs 15.6%; p < 0.0001) in the eyes with complications. Twenty-one children (21/142, 14.8%) with uveitis underwent a total of 62 ocular surgeries. Good visual acuity (20/40 or better) was found in 90.8% of eyes (159/175) and in both eyes of 87% of cases (94/108), impaired visual acuity in 6 eyes of 4 cases (6/175, 3.4%), and blindness in 10 eyes of 10 cases (10/175, 5.7%). Only 2 patients had reduced visual acuity in both eyes. Surgery was the single most important risk factor for reduced visual acuity at the last follow-up (p = 0.0086). Conclusions: Most uveitic eyes with JIA achieved good visual outcome despite uveitic complications. [Copyright &y& Elsevier]

Purpose: Evaluation of clinical aspects and cost-effectiveness of use of self-expanding metallic stents in the treatment of acute colonic obstruction as either a preoperative procedure or palliation., Materials and Methods: Thirteen consecutive patients, aged 49-83 years (mean, 67 years), with clinical and radiologic signs of colonic obstruction were treated as a preoperative procedure in 10 patients and as a palliative treatment in three. A total of 16 self-expanding metallic stents (diameter, 16 mm; length fully expanded, 56 mm) were implanted with combined fluoroscopic and endoscopic guidance. The costs (hospitalization, intensive care unit, stent placement, and surgery) were compared with costs for 13 surgically treated patients at the same hospital., Results: Stent placement was successful in 12 of the 13 patients; all recovered from mechanical obstruction, and single-stage surgery was possible in eight of nine patients treated preoperatively. One very narrow stenosis could not be passed. Dysfunction occurred in two long stenoses after 5 days with reocclusion 2 and 6 weeks, respectively, after stent placement. A cost reduction of 19.7% was observed as a result of shorter hospitalization and a lower complication rate. In patients with colon cancer in the preoperative treatment group, the cost reduction increased to 28.8%., Conclusion: Metallic stent placement in patients with acute colonic obstruction was a minimally invasive and cost-effective preoperative procedure that allowed single-stage surgery in most cases. Stent placement for palliation should be limited to patient with special indications.