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Background Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in a 1 000 000 children(1). Currently there is nearly no data published about the course of jSSc patients with standardized assessment. We report our date from Juvenile Scleroderma Inception cohort (JSSIC) with a follow up of 24 months. Objectives: to evaluate the organ involvement and patient reported outcomes (PRO) during the first 24 months in the JSIC Methods The JSSIC is a prospective multicenter registry of patients with jSSc, who fulfill the adult classification criteria(2), and presented the first non-Raynaud symptom before 16 years of age and were younger then 18 years at the time of inclusion in the JSSIC. Patients who were followed at least 24 months in the JSSIC, were evaluated. Results 52 patients were followed at least 24 months in the registry. 77% were female and 77% had diffuse subtype. 19% had overlap features. Mean disease duration at time of inclusion was 3.2 years. Mean age of at Raynaud’s onset was 8.8 years and the first non-Raynaud’s symptom 9.4 years. 85% received DMARDs at the time of inclusion and 96% after 24 months. 88% of the patients were ANA positive, 35% anti-Scl70 positive and 3% anticentromere positive. The mean modified skin score decreased from 14.3 to 12.6. The frequency of Raynaud’s stayed around 87%. The frequency of the nailfold capillary changes increased from 56% to 63%, but the frequency of active ulcerations stayed stable around 21%. The number of patients with FVC Several PROs improved significantly. Patient global disease activity (VAS 0-100) changed from 46 to 29 (p=0.002), patient global disease damage (VAS 0-100) from 46 to 28 (p=0.02) and patient Raynaud activity VAS 0-100) from 27 to 14 (p=0.009) as physician global disease activity (VAS 0-100) from 43 to 29 (p=0.021) and physician global disease damage from 46 to 28 (P=0.01). Conclusion Over the 24 months observation period patient and physician related outcomes improved significantly. Regarding organ involvement there was an increase in patients of pulmonary hypertension and joint contractures. References [1] Beukelman T, Xie F, Foeldvari I. Assessing the prevalence of juvenile systemic sclerosis in childhood using administrative claims data from the United States. Journal of Scleroderma and Related Disorders. 2018;3(2):189-90. [2] van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737-47. Disclosure of Interests Ivan Foeldvari Consultant for: Chugai, Novartis, Jens Klotsche: None declared, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Maria T. Tererri: None declared, Jordi Anton Grant/research support from: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Consultant for: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Speakers bureau: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Tadej Avcin: None declared, Rolando Cimaz: None declared, Mikhail Kostik: None declared, Maria Katsikas: None declared, Dana Nemcova: None declared, Maria Jose Santos: None declared, Cristina Battagliotti: None declared, Lillemor Berntson: None declared, Juergen Brunner: None declared, Liora Harel: None declared, Tilmann Kallinich Grant/research support from: Novartis, Speakers bureau: Sobi, Roche, Novartis, CLB, Kirsten Minden Consultant for: AbbVie, Monika Moll: None declared, Anjali Patwardhan: None declared, Kathryn Torok: None declared, Nicola Helmus: None declared

Background Patients with juvenile idiopathic arthritis (JIA) may have a different body composition associated with reduced muscle mass and increased fat mass [1]. They display decreased physical fitness, perform less strenuous physical activities, and spend more time sleeping than do healthy children. A lower level of physical activity is associated with deconditioning and functional deterioration, favoring an inactive lifestyle. The risk of overweight might be further increased by the glucocorticoid treatment. Objectives Since obesity can increase inflammatory processes, cause early atherosclerotic changes and promote metabolic disorders, the objectives were a) to determine the prevalence of overweight and obesity in children and adolescents with JIA, and b) to examine the association between overweight and health-related parameters in this population. Methods A cross-sectional analysis of physicians’ recorded body weights and heights of patients with JIA enrolled in the NPRD in the year 2016 was performed. Overweight was defined as BMI >90th sex- and age-specific percentile and obesity as BMI >97th percentile. For comparison with data from the general German population [2], patients aged 3 to 17 years were considered. A linear regression model was used to explore the association between overweight and both clinical as well as self-reported outcomes. Results In total, data from 6.860 children and adolescents with JIA (age 11.5 ± 4 years, disease duration 4.6 ± 3.6 years, 67% girls, 39% persistent oligoarthritis) were analyzed. Overweight was found in 14% (including 6% obesity) of JIA cases. Comparative data from the German general population report an overweight prevalence of 15% (including 6% obesity). In contrast to the general population, overweight rates in JIA differed between girls and boys (girls 14% vs. boys 16%, p Conclusion The prevalence of overweight and obesity in young patients with JIA is similar to that of children and adolescents in the general population. The overweight rate increases with age and is strongly associated with functional restrictions and treatment with glucocorticoids. The role of overweight in the long-term outcome of JIA is an issue that still needs to be addressed. References [1] Gronlund MM, et al. Juvenile idiopathic arthritis patients with low inflammatory activity have increased adiposity. Scand J Rheumatol 2014;43:488–92. [2] Schienkiewitz A, et al. Ubergewicht und Adipositas im Kindes- und Jugendalter in Deutschland. Journal of Health Monitoring 2018; 3:16–23. Acknowledgement The National Paediatric Rheumatological Database has been funded by the German Children Arthritis Foundation (Deutsche Kinder-Rheumastiftung), AbbVie, Pfizer and Chugai. Disclosure of Interests Florian Milatz: None declared, Jens Klotsche: None declared, Martina Niewerth: None declared, Nils Geisemeyer: None declared, Jana Horstermann: None declared, Gerd Ganser: None declared, Ivan Foeldvari Consultant for: Chugai, Novartis, Angelika Thon: None declared, Rainer Berendes: None declared, Markus Hufnagel: None declared, Toni Hospach Speakers bureau: Chugai, Roche, Novartis, Kirsten Minden Consultant for: AbbVie

Background Insights in pathogenesis and the availability of new biologic drugs have created requirements and an increasing interest for encouragement of physical activity (PA) as long-term treatment option in patients with juvenile idiopathic arthritis (JIA). A low level of PA in healthy individuals is related to a higher incidence of overweight and hypertension in later life. This low level of PA might even be more dangerous for children with JIA, as they also have elevated inflammatory parameters, perhaps increasing the risk of future cardiovascular diseases. Objectives Since children and adolescents with physical disabilities may have an increased risk for developing a sedentary lifestyle, the objective was to investigate if encouragement of PA in most German medical care settings has led to PA levels in JIA similar to that of healthy counterparts. Methods Data from children and adolescents with JIA recorded in the German National Paediatric Rheumatologic Database (NPRD) in the year 2017 were considered for the analyses. In accordance with the methodology used in the general population survey [1], the achievement of the WHO recommendations on PA for health was determined on the basis of self-reported outcomes in individuals aged 3 to 17 years. Patients met the WHO criteria if they stated to be physically active for at least 60 minutes per day. Results In 2017, the data from 5.918 patients (mean age 11.2 ± 4.1 years, female 67%, disease duration 4.6 ± 3.7 years, persistent oligoarthritis 42%) were available for evaluation. Almost 35% of patients aged 3 to 17 years met the recommended physical activity level (72% aged 3 to 6; 47% aged 7 to 10; 27% aged 11 to 13; 16% aged 14 to 17). In the general population, 26% fulfilled the WHO recommendations on PA (46% aged 3 to 6; 27% aged 7 to 10; 38% aged 11 to 13; 12% aged 14 to 17). In multivariable analyses, increasing age (OR 1.27; 95%CI: 1.24-1.29), psoriatic arthritis (OR 1.49; 95%CI: 1.06-2.11), overweight (OR 1.33; 95%CI: 1.04-1.71), functional disability (OR 0.83; 95%CI: 0.69-0.98), and worse patient-reported overall well-being (OR 1.10; 95%CI: 1.03-1.17) were associated with non-achieving the recommended PA amount. Conclusion Encouraging PA in most German medical care settings and the growing attention of the importance of regular PA for pleasure and health benefits may have led to a similar or even higher amount of PA compared to healthy counterparts. However, since a large proportion does not meet the global recommendations on PA, further research should address especially patients with inactive or minimal active disease who have previously largely refrained from PA. In order to derive adequate strategies, future work is warranted to comprehensively and objectively measure PA behavior in this population. References [1] Finger JD, et al. Korperliche Aktivitat von Kindern und Jugendlichen in Deutschland - Querschnittergebnisse aus KiGGS Welle 2 und Trends. Journal of Health Monitoring 2018;3:24–31. Acknowledgement The National Paediatric Rheumatological Database has been funded by the German Children Arthritis Foundation (Deutsche Kinder-Rheumastiftung), AbbVie, Pfizer and Chugai. Disclosure of Interests Florian Milatz: None declared, Martina Niewerth: None declared, Jana Horstermann: None declared, Nils Geisemeyer: None declared, Peter Haas Grant/research support from: Pfizer, Gerd Horneff: None declared, Tilmann Kallinich Grant/research support from: Novartis, Speakers bureau: Sobi, Roche, Novartis, CLB, Matthias Hartmann: None declared, Joachim Peitz: None declared, Josephine Merker: None declared, Kirsten Minden Consultant for: AbbVie

Background Since 2000, Etanercept is approved for polyarticular juvenile idiopathic arthritis (JIA). Subsequently, approval has been extended to additional JIA categories. Objectives To describe efficacy and safety of Etanercept in clinical practice in JIA patients in comparison to a biologic-naive JIA cohort using the German Biologics registry (BiKeR). Methods Baseline demographics and disease activity parameters were documented. Efficacy was determined using the JADAS10. Safety assessments were based on adverse events reports (AE) processed according to MedDRA. Results Until October 1, 2018, 2645 JIA patients treated with Etanercept were registered, representing 5820 patient-years (PY) of exposure. The total observation time (from date of first dose until last follow-up, censored, if another biologic was started) was calculated as 8080 PY. In general, the cohort treated with Etanercept had experienced disease duration of 4.2+/-3.7 years (mean+/-SD). 2303 patients (87%) were pretreated with methotrexate, 100 with alternative biologics. Concomitantly, 1822 patients (69%) received methotrexate, 2098 (79%) NSAIDs, 949 (36%) systemic corticosteroids as well as further drugs in lower numbers. In the control cohort, 1517 biologic naive JIA patients started methotrexate. At last follow-up, 68%/62%/50%/34% of patients reached JIAACR30/50/70/90 criteria. The median (IQR1-3) JADAS10 score decreased from 15.0 (10-20.4) at baseline to 3.5 (0.7-10.1). 60% of patients achieved a JADAS defined minimal disease activity, 40% reached a JADAS remission. 1924 AE were observed (33.1/100PY [95% CI 31.6-34.6]). 221qualified as SAE (3.8/100PY [3.3-4.3]). These figures were compared to 1292 AE reported during 3714PY in the control cohort (34.8/year [32.9-36.8]) and 52 SAE (1.4/100PY [1.1-1.8]). The most frequent SAE in the Etanercept cohort were grouped in the MedDRA SOC infections and infestations (n=54) followed by musculoskeletal and connective tissue disorders (n=27), eye disorders (n=21), gastrointestinal disorders (n=17) and nervous system disorders (n=15). There were 5 deaths. Serious infections were of bacterial and viral origin and mostly occurred once only. Adverse events of special interest were uveitis (n=109), H. zoster (n=24), chronic inflammatory bowel disease (n=19), pregnancies (n=4), depression (n= 11), malignancies (n=8), demyelination (n=2). Adverse Events of Special Interest (AESI) were in the MTX–cohort uveitis (n=52), H. zoster (n=4), chronic inflammatory bowel disease (n=1), depression (n= 2), malignancies (n=4). A total of 1606 patients (60.7%) discontinued treatment. Of these 638 (39.7%) discontinued due to remission, 565 (35.2%) due to lack of efficacy and 188 (11.7%) because of intolerance. Conclusion The current analysis adds to the established safety profile of Etanercept and demonstrates that the rate of SAEs is comparable and consistent with the overall AE profile in paediatric patients. As expected, infections are the most frequent SAE. Uveitis is likely associated with JIA. Of notice, few patients developed a chronic inflammatory bowel disease and some a malignancy. No new safety signals specific to the paediatric population were identified in this large cohort of JIA patients. References [1] Geikowski T, Becker I, Horneff G; German BIKER Registry Collaborative Study Group. Predictors of response to etanercept in polyarticular-course juvenile idiopathic arthritis. Rheumatology (Oxford). 2014 Jul;53(7):1245-9 Acknowledgement The authors acknowledge the support of the patients, parents and all contributors Disclosure of Interests Ariane Klein: None declared, Gerd Ganser: None declared, Michael Ruhlmann: None declared, Frank Dressler Paid instructor for: Abbvie, Pfizer, Novartis, Toni Hospach Speakers bureau: Chugai, Roche, Novartis, Kirsten Minden Consultant for: AbbVie, Ralf Trauzeddel: None declared, Boris Huegle: None declared, Sonja Mrusek: None declared, Ivan Foeldvari Consultant for: Chugai, Novartis, Jasmin Kuemmerle-Deschner Grant/research support from: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Consultant for: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Speakers bureau: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Frank Weller-Heinemann: None declared, Andreas Urban: None declared, Gerd Horneff: None declared

Background: Tocilizumab (TOC) is approved for treatment of polyarticular juvenile idiopathic arthritis (pJIA). Data out of clinical practice are limited. Objectives: Long-term surveillance of patients initiating TOC treatment compared to a cohort of patients initiating a comparator biologic using the BIKER-registry. Methods: Baseline parameters, efficacy and safety parameters were compared. Efficacy outcomes were JADAS10 and joint counts. Functional status was determined with the Childhood Health Assessment Questionnaire disability-index (CHAQ-DI). Safety was assessed by adverse events (AE) reports. Results: Until January 2020, 152 patients have been recruited to each cohort. Patients starting on TOC were older at treatment start (12.1 vs. 10.1 years (y); p Upon TOC a substantial response to treatment with a significant reduction in JADAS 10 from 16.8 to 3.4 (p While the total number of AE was comparable between the TOC cohort (58/100PY) and in the control cohort (54/100PY; RR 1.1; 95%CI 0.9-1.4), more serious AE (SAE) were reported with TOC (4.8/100PY compared to 1.4/100PY; RR 3.5; 95% CI 1-10.9). Medically important infections and uveitis events were documented at significantly lower frequency in the TOC- (0.8/100PY) than in the control cohort (4.1/100PY; RR 0.2; 95% CI 0.04-0.9). SAE with TOC were suicidal intent (n=3), depression (n=2), exacerbation of JIA, abscess, gastrointestinal infection, abdominal pain, colitis, bone surgery and fracture (n=1). SAE in the control cohort were depression, osteomyelitis, gastrointestinal infection and superinfected eczema (n=1). No significant differences regarding cytopenia and elevated transaminases were observed. No gastrointestinal perforation, no vascular event, no malignancy and no death occurred. Conclusion: The efficacy of tocilizumab is comparable to that of alternative biologics. Tolerability was acceptable. As Tocilizumab was given as a second-line biologic in the vast majority of patients, comparisons between the cohorts have to be interpreted carefully. Observation in the registry is ongoing. Disclosure of Interests: Ariane Klein Consultant of: Celgene, Toni Hospach: None declared, Frank Weller-Heinemann: None declared, Sandra Hansmann Consultant of: Advisory board Novartis Pharma, Jasmin Kuemmerle-Deschner Grant/research support from: Novartis, Sobi, Consultant of: Novartis, Sobi, Speakers bureau: Novartis, Sobi, Maria Fasshauer Consultant of: Shire, CSL Behring, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Ivan Foeldvari Consultant of: Novartis, Christoph Rietschel Consultant of: Pfizer, Abbvie, Novartis, Chugai, and Sobi, Tobias Schwarz: None declared, Ralf Trauzeddel: None declared, Markus Hufnagel: None declared, Dirk Foell Grant/research support from: Novartis, Sobi, Pfizer, Speakers bureau: Novartis, Sobi, Rainer Berendes: None declared, Gundula Boeschow: None declared, Prasad Oommen Consultant of: Novartis, Frank Dressler: None declared, Astrid Helling-Bakki: None declared, Gerd Horneff Grant/research support from: AbbVie, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche

Background: Tocilizumab (TCZ) administered intravenously (IV) was effective for the treatment of polyarticular (p)JIA and systemic (s)JIA.1,2 Objectives: To evaluate the long-term safety and efficacy of subcutaneous (SC) TCZ in patients (pts) with pJIA or sJIA enrolled in a long-term extension (LTE) phase of two 52-week, open-label studies. Methods: Pts aged 1-17 years received body weight (BW)–based TCZ SC: pJIA pts who failed/could not tolerate MTX received TCZ 162 mg every 3 weeks for BW Results: Most pJIA (n=44) and sJIA (n=38) pts were female (72.7% and 55.3%) and white (88.6% and 84.2%); median (range) age was 9.0 (2-18) years. AE rates (Table) were similar regardless of BW. Most AEs were grade 1 or 2; grade ≥3 AEs were reported by 10/44 (20.8%) pJIA pts and 4/38 (10.5%) sJIA pts, most commonly nasopharyngitis (pJIA, 17/44 [38.6%]; sJIA, 11/38 [28.9%]). Other AEs reported in ≥15% of pts included arthralgia, gastroenteritis, cough, vomiting, diarrhea, pyrexia, headache, and oropharyngeal pain (pJIA) and upper respiratory tract infection, cough, pyrexia, arthralgia, and rash (sJIA). No opportunistic infections developed. Neutropenia AEs were reported by 6/44 (13.6%) pJIA pts and 7/38 (18.4%) sJIA pts. SAEs occurred in 5/44 (11.4%) pJIA pts (furuncle, appendicitis, pneumonia, eye pain/headache, infectious mononucleosis) and 2/38 (5.3%) sJIA pts (pneumonia, craniocerebral injury from a fall); only pneumonia (pJIA) was considered treatment related. Neutralizing anti-TCZ antibodies developed in 2 (4.7%) pJIA pts and no sJIA patients. No deaths were reported in the LTE study. Conclusion: In this LTE study in children with pJIA or sJIA, SC TCZ continues to have an acceptable tolerability profile with no new safety concerns. References: [1] Brunner HI et al. Ann Rheum Dis. 2015;74:1110-1117. [2] 2. De Benedetti F et al. N Engl J Med. 2012;367:2385-2395. Disclosure of Interests: Fabrizio De Benedetti Grant/research support from: Abbvie, SOBI, Novimmune, Roche, Novartis, Sanofi, Pfizer, Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (> 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties., Consultant for: Received honoraria for consultancies or speaker bureaus (

Background Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There are limited data regarding the clinical presentation of jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is a multinational registry that prospectively collects information regarding patients with this disease in a standardized manner. Objectives Evaluation of the jSSc patients at the time of inclusion in the JSSIC. Methods Patients were included in the JSSIC if they fulfilled the adult classification criteria, if they presented the first non Raynaud symptom before 16 years old and if they were younger than 18 years of age at the time of inclusion. Patients’ characteristics at time of inclusion were evaluated. Results Currently, the cohort includes 120 patients, being 89% Caucasian and 80% female. The majority had diffuse subtype (74%) and 18% had overlap features. The mean age of onset of Raynaud phenomenon was 9.7 years in the diffuse subtype (djSSc) and 10.7 years in the limited subtype (ljSSc). The mean age of non-Raynaud’s symptoms was 10.0 years in the djSSc and 11.4 years in the ljSSc (p=0.041). Mean disease duration at time of inclusion was 3.4 years in the djSSc and 2.4 years in the ljSSc group. Mean Modified Rodnan skin score was 17.5 in the djSSc and 7.3 in the ljSSc (p=0.002). Gottron papulae were significantly more common in the djSSc compared to ljSSc group (29% vs 6%, respectively) (p=0.011). History of ulceration was significantly more common in the djSSc than in the ljSSc group (57% vs 30%, respectively) (p=0.004). FVC Conclusion In this large cohort of jSSc patients there were surprisingly not many significant differences between djSSc and ljSSc. According to the physician global scores the djSSc patients have a significantly more severe disease. Disclosure of Interests Ivan Foeldvari Consultant for: Chugai, Novartis, Jens Klotsche: None declared, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Valda Stanevicha: None declared, Flavio R. Sztajnbok: None declared, Maria T. Tererri: None declared, Ekaterina Alexeeva: None declared, Jordi Anton Grant/research support from: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Consultant for: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Speakers bureau: Grant/research support, consultant or speakers bureau from AbbVie, Alexion, Bristol-Myers Squibb, ChemoCentryx, Gebro, GlaxoSmithKline, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Maria Katsikas: None declared, Vanessa Smith: None declared, Tadej Avcin: None declared, Rolando Cimaz: None declared, Mikhail Kostik: None declared, Thomas Lehman: None declared, Walter Alberto Sifuentes-Giraldo: None declared, Simone Appenzeller: None declared, Mahesh Janarthanan: None declared, Monika Moll: None declared, Dana Nemcova: None declared, Maria Jose Santos: None declared, Dieneke Schonenberg: None declared, Cristina Battagliotti: None declared, Lillemor Berntson: None declared, Blanca Bica: None declared, Juergen Brunner: None declared, Patricia Costa Reis: None declared, Despina Eleftheriou: None declared, Liora Harel: None declared, Gerd Horneff: None declared, Tilmann Kallinich Grant/research support from: Novartis, Speakers bureau: Sobi, Roche, Novartis, CLB, Dragana Lazarevic: None declared, Kirsten Minden Consultant for: AbbVie, Susan Nielsen: None declared, Farzana Nuruzzaman: None declared, Anjali Patwardhan: None declared, Yosef Uziel: None declared, Nicola Helmus: None declared

Background Etanercept is the most frequently used biologic drug in patients with JIA. Children and adolescents with JIA are treated with etanercept often over long periods of time, sometimes even into adulthood. The knowledge about its long-term safety, especially with regard to the occurrence of new-onset immune-mediated diseases and malignancies, is still limited. Objectives To investigate the exposure-adjusted rates of adverse events (AE) and serious AE (SAE) in patients with JIA with long-term use of etanercept. Methods Patients with JIA who were enrolled in the German biologic register BiKeR and were born before 30.05.2000 (at least 18 years of age at this date) were considered for this analysis. The follow-up register JuMBO ensures the long-term follow-up into adulthood. An AE or SAE was attributed to etanercept when the treatment was either ongoing or terminated in less than 3 month prior to the event. The incidence of malignancies was estimated in patients who were ever exposed to etanercept with at least one dose. Results Of 4546 patients who were currently enrolled in BiKeR a total of 2584 JIA patients were eligible for the JuMBO register (>18 years). Among those, 1765 (68%) were ever exposed to etanercept and observed for a mean of 6.8±4.9 years (including 1101 into adulthood). The majority of them had polyarthritis (35%), followed by enthesitis-related arthritis (20%) and extended oligoarthritis (17%). The patients were exposed to etanercept for a total of 4.2 years (mean, 6,726 exposure years, EY); 518 patients were continuously treated with etanercept for at least 5 years (4,534 EY). In total, 124 autoimmune events (1.84/100EY) and 7 other immune disorders (0.10/100EY) were reported in 102 (5.8%) and 7 (0.4%) patients with onset on average 10.2 years after JIA onset, and 6.8 years after start with etanercept, respectively. The number of selected immune-mediated events and the exposure-adjusted rates are given in the table. In addition, 11 malignancies (0.10 events/100 person-years) were reported in patients ever exposed to etanercept. Among those (mean age at onset 20.3 years), 4 malignancies were reported in childhood and 7 in young adulthood. Three patients were also exposed to other biologics before the incidence of malignancy. The malignancies occurred on average 12.1 years after JIA onset, 10.4 years after start with a first DMARD and 7.5 years after first exposure to etanercept. Conclusion This is the first long-term large safety study in prospectively followed JIA patients with focus on new-onset immune-mediated diseases and malignancies in etanercept. The study also highlights that it is important to prospectively collect data on adverse events under treatment with biologics in JIA, in particular with respect to the risk of malignancies in young adulthood. Disclosure of Interests Jens Klotsche: None declared, Ariane Klein: None declared, Martina Niewerth: None declared, Gerd Ganser: None declared, Peer Aries: None declared, Marisa Walther: None declared, Peter Haas Grant/research support from: Pfizer, Gernot Keyser: None declared, Gerd Horneff: None declared, Kirsten Minden Consultant for: AbbVie

Background Timely access to specialized and multidisciplinary care is a core principle in the management of JIA patients and crucial to achieve the best possible outcome for children and adolescents with JIA. Objectives To assess satisfaction with access to specialized care and used health care services of families with children with JIA and to identify factors associated with unmet needs. Methods Parents of JIA patients enrolled in the early JIA cohort study ICON completed the Child Healthcare Questionnaire on Satisfaction, Utilisation and Needs (CHC-SUN)1 and the Family Burden Questionnaire (FaBel) 3 months after enrolment. The socioeconomic status, disease activity, patients’ functional ability and quality of life were also assessed. Factors associated with unmet needs were identified by logistic regression analysis. Results Data were available from 835 families with children diagnosed with JIA 3 months (median, IQR 1-6) after symptom onset and cared for by paediatric rheumatologists (68% females). At assessment (4.6 months after diagnosis, median), 67% of patients received non-steroidal anti-inflammatory drugs, 50% received conventional synthetic DMARDs and 8% biologic DMARDs. In addition, the following health care services were utilized: 84% physiotherapy, 23% occupational therapy, 21% supply with physical aids, 17% telephone counselling, 15% health education, 13% social worker services, and 11% psychological counselling. Unmet needs were most frequently reported for health education (19%), rehabilitation services (11%), psychological counselling (11%), self-help groups (10%), and lowest for physiotherapy (2%). Unmet needs varied depending on the type of service and JIA category (Table). They were more frequently observed in families with higher burden as indicated by FaBel, but were not associated with migration background and socioeconomic status. Most parents were generally satisfied with their child’s health care (satisfied 30%, very satisfied 42%, extremely satisfied 23%). Satisfaction was highest with the behaviour of doctors and lowest with school-related services (e.g. 36% not or partly satisfied with teachers’ knowledge about the child’s illness) and diagnosis/information (e.g. 29% not or partly satisfied with the time required for the diagnosis). Conclusion There are, although infrequently, unmet needs and dissatisfaction with health services among families with children who have JIA and receive specialized care. Whether these deficits are relevant for long-term JIA outcomes will be further investigated in the ICON study. Reference [1] Schmidt S, Thyen U, Chaplin J, Mueller-Godeffroy E; European DISABKIDS Group. Cross-cultural development of a child health care questionnaire on satisfaction, utilization, and needs. Ambul Pediatr. 2007;7:374-82. Acknowledgement Funding by Federal Ministry of Research: 01 ER 1504A Disclosure of Interests Michaela Heinrich: None declared, Jens Klotsche: None declared, Claudia Sengler: None declared, Martina Niewerth: None declared, Frank Weller-Heinemann: None declared, Peter Haas Grant/research support from: Pfizer, Gerd Horneff: None declared, Toni Hospach Speakers bureau: Chugai, Roche, Novartis, Kirsten Moenkemoeller: None declared, Angelika Thon: None declared, Ivan Foeldvari Consultant for: Chugai, Novartis, Kirsten Minden Consultant for: AbbVie

Background Anakinra (ANA) is recommended for treatment of systemic juvenile idiopathic arthritis (sJIA) and has recently received EMA approval. Objectives To describe safety and effectiveness of ANA in clinical practice in sJIA patients documented in the German Biologics registry (BiKeR). Methods Demographic and clinical parameters of the patients were recorded at BiKeR enrollment, ANA effectiveness was evaluated by half-yearly evaluation of disease activity using JADAS-10. Safety assessments were based on MedDRA-coded adverse events (AE) reports. Results Until December 1 2018, 50 sJIA patients treated with ANA were registered, representing 113.4 patient-years (PY) of observation, with 10 patients (20%) receiving ANA for at least 4 years. The cohort treated with ANA had experienced a prior disease duration of 3.5+/-3.8 years (mean+/-SD). Most sJIA patients treated with ANA received pretreatment: 35 patients (70%) were pretreated with at least one other biologic, mostly etanercept (n=28), followed by tocilizumab (6) and canakinumab (1). Further pretreatments included methotrexate (MTX, n= 35), ciclosporine A (n=13), azathioprine (n=8) and other DMARDs at lower frequency. 39 patients had received corticosteroids. Concomitant treatment consisted of NSAIDs (n=20), systemic steroids (n=35), MTX (n=29) and other DMARDs (n=8). At month 3, JADAS minimal disease activity (MDA)/JADAS remission and inactive disease according to Wallace [1] were observed in 42%/37%and 47%, respectively. After one year of treatment, the rates were 62%/45% and 56%, respectively (Figure 1). Conclusion The current analysis adds to the established safety profile of Anakinra and demonstrates that in ANA treated patients with sJIA the rate of SAEs was comparable and consistent with the overall AE profile of ANA in pediatric patients. Hospitalisation was the usual reason for classification as serious AE. No new safety signals specific to the paediatric population were identified in this large cohort of JIA patients. Disclosure of Interests Ariane Klein: None declared, Veronika Ntam Atemnkeng: None declared, Frank Dressler Paid instructor for: Abbvie, Pfizer, Novartis, Ivan Foeldvari Consultant for: Chugai, Novartis, Rolf Michael Kuster: None declared, Toni Hospach Speakers bureau: Chugai, Roche, Novartis, Gerd Ganser: None declared, Kirsten Minden Consultant for: AbbVie, Dirk Foell Grant/research support from: not specified, Consultant for: not specified, Speakers bureau: not specified, Frank Weller-Heinemann: None declared, Jasmin Kuemmerle-Deschner Grant/research support from: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Consultant for: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Speakers bureau: Jasmin Kuemmerle-Deschner is an employee of University of Tuebingen, Germany, and received consultants/speakers fees from Novartis and SOBI pharmaceuticals and grant support from SOBI and Novartis., Boris Huegle: None declared, Christoph Rietschel: None declared, Eggert Lilienthal: None declared, Gerd Horneff: None declared

Background Efficacy of SC abatacept (ABA) in patients (pts) with polyarticular-course juvenile idiopathic arthritis (pJIA) was shown in a 2-year, open-label, Phase III international study (NCT01844518).1 Restricted growth and delayed puberty are associated with pJIA;2 however, the effect of ABA on growth and development is unknown. Objectives To investigate the effect of ABA on height, BMI and Tanner staging, and to assess the impact of BMI on ABA efficacy in pts with pJIA. Methods Patients with pJIA were grouped into two age cohorts (2–5 and 6–17 years [yrs]) and received weight-tiered SC ABA (10 to −2 to ≤1), overweight (>1 to ≤2) and obese (>2) (intent-to-treat, non-responder imputation for missing efficacy data). In the 6–17-yr cohort, Tanner staging was recorded at BL and at discontinuation or completion of study. Results Data for standardised height and BMI are presented. In 6–17-yr-old pts with BL Tanner stage ≤3 (n=100), BL mean (SD) height was −0.53 (1.53); at D645, mean (95% CI) change was 0.20 (0.06, 0.34; Figure 1). In 6–17-yr-old pts with a BL height ≤−1 (n=42), BL mean (SD) height was −2.10 (1.01) and mean (95% CI) change at D645 was 0.49 (0.23, 0.76). In 2–5-yr-old pts, BL mean (SD) height was 0.07 (1.37); at D645, mean (95% CI) change was 0.13 (−0.09, 0.36). In 13 pts aged 6–17 yrs of the lowest height (Tanner stage ≤3 and BL height ≤−2), mean (SD) height increased from −3.28 (1.10) at BL to −2.59 (1.31) by D645. At BL, mean (SD) BMI was 0.20 (1.33) in the 6–17-yr cohort (n=173) and 0.25 (1.57) in the 2–5-yr cohort (n=46); at D645, mean (95% CI) change in BMI was 0.02 (−0.13, 0.16; Figure 2) and 0.17 (−0.08, 0.42), respectively. Overall, no relationship was seen between change from BL in Juvenile Arthritis Disease Activity Score 71 (JADAS71) and BL BMI in either age cohort. The proportions (odds ratio [OR]; 95% CI of OR) of obese pts aged 6–17 yrs achieving JIA-ACR30 (0.15; 0.04, 0.55), JIA-ACR50 (0.18; 0.05, 0.63), JIA-ACR70 (0.17; 0.04, 0.67) and JADAS71 minimal disease activity (0.27; 0.07, 1.03) at Day 645 were significantly lower compared with pts with a normal BMI, but the sample size in the obese category was low (n=12); this effect was not seen in overweight pts (n=37). In female pts (n=92), shifts in Tanner stages from BL to D645 were as expected; male sexual maturation was difficult to assess due to the small sample size (n=20). Conclusion Abatacept improves growth and development in pts with pJIA. Early therapeutic intervention with abatacept may help reduce growth restrictions in pts with pJIA. BMI may impact the efficacy response, resulting in lower efficacy in obese, but not in overweight, pts with pJIA versus pts with normal BMI. References [1] Brunner HI, et al. Arthritis Rheumatol2018;70:1144–54. [2] Umlawska W, et al. Arch Med Sci2010;6:19–23. Acknowledgement Professional medical writing: Lola Parfitt, MRes, Caudex; funding: Bristol-Myers Squibb Disclosure of Interests Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (> 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties., Consultant for: Received honoraria for consultancies or speaker bureaus ( I perform consultancy activities on behalf of the Gaslini Institute for the companies listed below: AbbVie, Biogen, Boehringer Ingelheim, Bristol-Myers Squibb, EMD Serono, Janssen, Novartis, Pfizer, R-Pharm. The money received for these activities are directly transferred to the Gaslini Institute’s bank account. Before March 2016, I was the head of the Pediatric Rheumatology Department at the G. Gaslini Hospital, where the PRINTO Coordinating Centre is located. For the coordination activity of the PRINTO network, the Gaslini Hospital received contributions from the industries listed in this section. This money has been reinvested for the research activities of the hospital in fully independent manners besides any commitment with third parties., Daniel J Lovell Consultant for: Consulting fees and/or honoraria from Astra Zeneca, Wyeth Pharma, Amgen, Abbott, Pfizer, F. Hoffmann-La Roche, Novartis, UBC, Takeda, GSK, Boehringer, and Celgene