V. Gerloni

SAT0489 Disease Activity and Health Status in a Cohort of Young Adults with JIA

Background Juvenile Idiopathic Arthritis (JIA) is the most prevalent chronic rheumatic disease in childhood. Disease activity can persist for many years, even through adulthood. Objectives To evaluate disease activity and health status in a cohort of young adults with JIA. Methods In order to identify patients with persistent active disease or in Clinical Remission off medication (CR) according to Wallace criteria all young people (over 18 year old), followed in our Transitional Care Clinic, were assessed by clinical examination [including registration of number of joints with swelling, tenderness and limited range of motion (LROM), number of active joints] and laboratory parameters. Physicians global assessment of disease activity measured on a 10 cm VAS, Quality of life according to the Medical Outcome Study 36-item Short Form Health Survey (SF-36), physical disability and discomfort by the Health Assessment Questionnaire (HAQ) were also recorded. Results 335 consecutive JIA pts (F 237) were evaluated: 39 (11.6%) systemic arthritis, 117 (34.9%) persistent oligoarticular, 48 (14.3%) extended oligoarticular, 80 (23.8%) polyarticular JIA (21 were RF positive), 37 ERA (11%), 14 psoriasic JIA (4.1%). Median age at disease onset was 7.0 yrs (mean 7.8, range 0.1-16.0). Median disease duration was 21.9 yrs (mean 22.7, range 7-51). 237/335 pts were on antirheumatic medication: 134 with biological therapy (BT), 167 with one or more DMARDs and 125 with NSAIDs. Out of the 134 treated with BT 90 were also treated with one or more DMARDs. 98 pts were off medication: 66 of them (67.3%) were inactive and 73 had physicians VAS <1 cm. In total 40.2% (135/335) presented active joints, ranging from 48.6% (12/39) systemic JIA to 28.5% (4/14) psoriasic JIA. All 335 pts received also SF-36 physical score (median 56, mean 62, range 1-99) and SF-36 mental score (median 61, mean 623, range 12-100). In total 149 pts (44.4%) had a pathological HAQ ranging from 69.2% of systemic onset JIA (median 0.625, mean 0.8, range 0-3) to 29% of persistent oligoarticular JIA (median 0.0, mean 0.2, range 0-1.6). Conclusions Our results support that a substantial proportion of the young adults with JIA had an active disease or was on CRM (still needing antirheumatic therapy) and patient-reported health status was not completely satisfactory in a significant number of them. These results suggest that JIA is not a self-limiting disease and therefore ideally all young pts should be followed by both pediatric and adult rheumatology as a part of a transitional care program. References Selvaag AM, et al. Disease progression into adulthood and predictors of long-term active disease in juvenile idiopathic arthritis. Ann Rheum Dis 2014;0:1-6. Disclosure of Interest None declared

FRI0356 Clinical remission in 291 juvenile idiopathic arthritis patients treated with biological agents

Objectives determine response to biological therapy (BT) in Juvenile Idiopathic Arthritis (JIA) pts in term of clinical remission on medication (CRM) according to Wallace criteria, and to identify baseline factors predictors of CRM, in a 10-year experience in a single centre Methods In our Pediatric Rheumatology Unit, each pt enrolled in BT is prospectively assessed at start of treatment and then every 3 mo for evaluation of safety and efficacy according to ACRpedi30 criteria. All clinical charts of JIA pts who started BT between Nov’99 and Jul’10 has been retrospectively reviewed to characterize disease activity by applying definition of inactive disease (ID), clinical remission on medication (CRM), clinical remission off medication (CR) according to Wallace criteria Results 301 JIA pts, non-responders or intolerant to DMARDs and treated with one or more BT, were identified. Median disease duration, from onset to the beginning of 1st BT, was 7.9 yrs (mean 9.7; range 0.2-41.4). Out of 301 enrolled pts, 291 were eligible for end point of interest. 72.1% were female. According to ILAR criteria, 131 had oligoarthritis (47 persistent and 84 extended), 58 polyarthritis RF neg, 25 polyarthritis RF pos, 50 systemic disease, 17 Enthesitis Related Arthritis and 10 psoriatic arthritis. Median age at onset was 5.2 yrs (mean 6.7; range 0.5-16.8). In total 569 cycles of BT (490 anti-TNF, 79 anti-cytokines and anti-cells treatments) were done. Mean n° of BT cycles for each pt was 1.9 (range 1-8). Considering only 1st cycle of BT, at last observation, 134 pts obtained at least one period of CRM of variable duration. Only 4 pts reached CR and 6 pts reached CR off BT, continuing only DMARDs. Median treatment duration to reach the CRM was 12 mo. CRM was achieved in 169 of 569 treatments. The chance to reach CRM is strictly related to 1st BT course: 134 of 169 successful treatments (78.6%) were 1st BT courses, and 134 of 291 1st BT courses, vs 35 of 298 subsequent BT courses, led to CRM. Multivariate statistical analysis showed that probability of CRM with 1s BT course is not significantly associated with ILAR categories, drug used and age at onset of disease (p>0.05), is positively correlated with male gender (p=0.04), is negatively correlated with disease duration before starting BT (p=0.06). Concomitant administration of DMARDs raised the chance of reaching CRM (p=0.02) Conclusions Upon treatment with the 1st biological agent, many children and young JIA pts, non-responders to DMARDs, reached at least one sustained period of CRM, although they had suffered from long-standing, refractory disease. Earlier initiation of therapy and concomitant DMARDs seems increase chance of reaching remission. Nevertheless persistent CR off medication remains a rare event, but according to Wallace criteria, definition of CR is “12 mo of ID after the end of all treatments”, therefore possibility to reach CR depends primarily on when (and if) you decide to stop all efficacious treatments Disclosure of Interest None Declared

Chapter 18 TNF-Inhibitors in Pediatric Rheumatology

Publisher Summary This chapter discusses the pathogenesis and adverse events of tumor necrosis factor (TNF)inhibitors in pediatric rheumatology. The overproduction of the inflammatory cytokine TNFα plays a key role in the maintenance of many chronic inflammatory rheumatic diseases, particularly those that depend on relationship between T-cells and macrophages. TNFα induces bone reabsorption, to inhibit proteoglycan and collagen synthesis to induce prostaglandin E 2 and collagenase release from synovial cells, and to stimulate fibroblast growth. TNFα also plays a pivotal role in the enhancement of inflammatory cell trafficking into synovium, by regulating the expression of adhesion molecules on the endothelial cells. The clinical experience on TNFα inhibition in juvenile idiopathic arthritis (JIA) is mainly limited to Etanercept is the only TNFα inhibitor approved until now for use in children with active polyarticular JIA despite prior methotrexate (MTX) therapy. Infliximab and adalimumab are used in the context of open label trials and in two large controlled studies.