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BackgroundJuvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children (1). In adult patients there are significant differences between the clinical presentation of diffuse and limited subtypes (2). We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort up to 2021.ObjectivesTo study the clinical presentation of jSSc patients with diffuse (djSSc) and limited (ljSSc) subtypes.MethodsWe reviewed the clinical baseline characteristics of the patients, who were recruited to the juvenile scleroderma inception cohort (jSScC) (3, 4) till 1st of December 2021. jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion.Results210 patients with jSSc were included in the cohort, 71% (n=162) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3 – 12.9) and the median age at the first non-Raynaud symptom was 10.9 years (7.4 – 13.2). Median disease duration was 2.5 years (1 – 4.4) at the time of inclusion. The female/male ratio was significantly lower in the djSSc subtype (3.7:1 versus 5:1, p< -2 z score (20% versus 4%, p=0.003) and decreased joint range of motion (64% versus 46%, p=0.019). Patients with ljSSc had significantly higher rate of cardiac involvement (13% versus 2%, p=0.001).Regarding patient related outcomes djSSc patients had more severe disease, looking at patient reported global disease activity (VAS 0 – 100) (40 versus 25, p=0.039), patient reported global disease damage (VAS 0 – 100) (40 versus 25, p=0.021) and patient reported assessment of ulceration activity (10 versus 0, p=0.044). Regarding physician related outcomes the physician reported global disease activity (VAS 0 – 100) (32 versus 20, pConclusionIn this jSSc cohort, the largest in the world, djSSc patients have a significantly more severe disease than ljSSc patients. Interestingly, we found no differences regarding interstitial lung disease and pulmonary hypertension.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]Dougherty DH, Kwakkenbos L, Carrier ME, Salazar G, Assassi S, Baron M, et al. The Scleroderma Patient-Centered Intervention Network Cohort: baseline clinical features and comparison with other large scleroderma cohorts. Rheumatology (Oxford). 2018;57(9):1623-31.[3]Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, et al. Differences sustained between diffuse and limited forms of juvenile systemic sclerosis in expanded international cohort. www.juvenile-scleroderma.com. Arthritis Care Res (Hoboken). 2021.[4]Foeldvari I, Klotsche J, Torok KS, Kasapcopur O, Adrovic A, Stanevica V, et al. CHARACTERISTICS OF THE FIRST 80 PATIENTS AT TIMEPOINT OF FIRST ASSESSMENT INCLUDED IN THE JUVENILE SYSTEMIC SCLEROSIS INCEPTION COHORT. WWW.JUVENILESCLERODERMA.COM. Journal of Scleroderma and Related Disorders. 2018;4(1-13).Disclosure of InterestsNone declared

BackgroundJuvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children (1). The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects longitudinal data prospectively in jSSc, allowing the evaluation of the development of organ involvement and patients and physician reported outcomes in jSSc over time.ObjectivesTo review the changes in the clinical characteristics and patient and physician reported outcomes over 12 months observation period from the time of inclusion into the cohort.MethodsThe jSScC cohort enrolls jSSc patients who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion (2, 3). We reviewed jSScC patient clinical data and patient and physician reported outcomes, who had 12 months follow up from the time of inclusion until 1st of December 2021.ResultsWe could extract data of 113 patients. The female/male ratio was 3.5:1. Median age of onset of Raynaud´s was 10.1 years and the median age of onset of non-Raynaud´s was 10.8 years. Eighty-eight percent of the patients were treated with disease modifying anti-rheumatic drugs (DMARDs) at time of inclusion in the cohort (T0) and 93% after 12 months (T12). Median disease duration was 2.5 years at T0. Antibody profile stayed unchanged. Only 3 clinical parameters changed and improved significantly, the median modified Rodnan skin score improved from 13 to 8 (p=0.002), the number of patients with swollen joints decreased from 17% to 8% (p=0.043) and number of patients with joints with pain on motion decreased from 20% to 12% (p=0.048). All other organ involvement did not show any statistically significant change from T0 to T12.All collected patient reported outcomes improved significantly from T0 to T12: the patient reported disease activity (VAS 0 – 100) from 40 to 20 (p=0.011), the patient reported disease damage (VAS 0 – 100) from 40 to 20 (p=0.001), patient reported ulceration activity (VAS 0 – 100) from 10 to 0 (p=0.02) and the CHAQ score from 0.3 to 0.1 (p=0.002). Two of the three physician reported outcomes improved significantly, the physician global disease activity (VAS 0 – 100) from 30 to 20 (p=0.011) and physician reported global disease damage (VAS 0 – 100) from 30 to 25 (p=0.028).ConclusionSkin and musculoskeletal clinical features improved over 12 months, with almost all patients on DMARDs, supporting likely response of these features to therapy. It was promising that internal organ involvement, like cardiac and lung, although potentially stable, did not significantly worsen or increase. The most striking observation in the positive direction is improvement across several patient and physician reported outcome measures over the 12 month time period in this large international cohort.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]Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, et al. Differences sustained between diffuse and limited forms of juvenile systemic sclerosis in expanded international cohort. www.juvenile-scleroderma.com. Arthritis Care Res (Hoboken). 2021.[3]Foeldvari I, Klotsche J, Torok KS, Kasapcopur O, Adrovic A, Stanevica V, et al. CHARACTERISTICS OF THE FIRST 80 PATIENTS AT TIMEPOINT OF FIRST ASSESSMENT INCLUDED IN THE JUVENILE SYSTEMIC SCLEROSIS INCEPTION COHORT. WWW.JUVENILESCLERODERMA.COM. Journal of Scleroderma and Related Disorders. 2018;4(1-13).Disclosure of InterestsNone declared

BackgroundJuvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently no medications are licensed for the treatment of jSSc. Due to its rarity, only recently have the first management and treatment guidelines been published, the jSSc SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) recommendations, reflecting consensus opinion upon pediatric rheumatologists (1).ObjectivesTo better understand treatment practices internationally for jSSc, both at baseline and over 24 months observation period and to compare if real world therapies are congruent with the recent SHARE recommendations.MethodsThe juvenile systemic sclerosis inceptions cohort (jSScC) is a multinational cohort that prospectively collects clinical data, including medications at baseline and subsequent visits. The jSScC enrollment criteria include age of onset of the first non-Raynaud symptom younger than 16 years and age younger than 18 years at cohort entrance. The frequency of medications (general category and specific medication) was calculated across the cohort at timepoint 0 (enrollment), 12 months and 24 months.ResultsWe extracted data from the jSScC of patients who were followed for 12 or 24 months. 109 patients were followed at time point 0 (T0) and 12 months (T12), and data was available for 77 of them up at 24 months (T24). The mean age of the patients was 13.2 years at the timepoint 0. 77% were female and 75% had diffuse subtype. Disease duration at baseline visit was 3.1 years. The medications the patients were on recorded by the physician were captured at T0, T12 and T24 listed in Table 1.Table 1.MEDICATIONSTime point 0N=109T12 monthsN=109T24 months N=77Any Medication92% (100)97% (106)97% (75)Vascular medications Endothelial receptor antagonist16% (17)24% (26)21% (16) PDE-5-Blocker5% (5)8% (9)9% (7)ImmunomodulatorsCorticosteroids52% (57)44% (48)44% (21)All csDMARDs:81% (88)93% (101)92% (71) csDMARDs monotherapy61% (67)66% (72)60% (46) csDMARDs combination therapy17% (18)15% (16)14% (11) Methotrexate51% (56)50% (55)39% (30) Mycophenolate Mofetil26% (28)44% (48)47% (36) Hydroxychloriquine11% (12)15% (16)21% (16) Cyclophosphamide12% (13)2% (2)1% (1) Azathioprine2% (2)2% (2)3% (2)All bDMARDs:5% (5)14% (15)18% (14) bDMARDs monotherapy2%(2)2%(2)1% (1) bDMARDs combined with csDMARDs3% (3)12% (13)17% (13) Tocilizumab2% (2)10% (11)14% (11) Rituximab2% (2)4% (4)4% (3) Adalimumab1% (1)0% (0)0% (0)Autologous Stem cell transplantation0% (0)1% (1)0% (0)csDMARDs: Conventional synthetic disease-modifying antirheumatic drugsb DMARDs: Biological disease-modifying antirheumatic drugsConclusionAt baseline half of the patients were on corticosteroids. This is more frequent than typical adult SSc practice but coincides with jSSc SHARE treatment recommendations (#1). After 12 months observation in the cohort over 90% of patients received a DMARD therapy. Methotrexate and mycophenolate mofetil were the most commonly prescribed DMARDs, which also reflects the SHARE treatment recommendations (#2, #3). At 12 months the use of glucocorticoid decreased and the use of bDMARDs increased. In general, biological DMARDs are typically considered in severe or refractory (SHARE recommendation #7), reflecting the lower percentage compared to csDMARDs. Autologous stem cell transplantation was observed in one patient at 12 months, reflecting an option in jSSc with progressive and refractory disease (SHARE recommendation #8). Endothelial receptor antagonists, such as bosentan, were used over time in approximately 20% of the patients, reflecting SHARE recommendation #6 for pulmonary hypertension and/or digital tip ulcers. This is the first evaluation looking at clinical medication practice pattern in jSSc, and its comparison to recently published consensus guidelines.References[1]Foeldvari I, Culpo R, Sperotto F et al. Consensus-based recommendations for the management of juvenile systemic sclerosis. Rheumatology (Oxford). 2021;60(4):1651-8.Disclosure of InterestsNone declared

Background:Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 per 1 000 000 children. There are limited data regarding the clinical presentation of jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is the largest multinational registry that prospectively collects information about jSSc patients.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 ACR/EULAR classification criteria for systemic scleroderma, if they presented the first non-Raynaud symptom before 16 years of age and if they were younger than 18 years of age at time of inclusion. Patients’ characteristics at time of inclusion were evaluated.Results:Until 15thof December 2019 hundred fifty patients were included, 83% of them being Caucasian and 80% female. The majority had the diffuse subtype (72%) and 17% of all jSSc had overlap features. The mean age of first presentation of Raynaud´s phenomenon was 9.8 years in the diffuse subtype (djSSc) and 10.7 years in the limited subtype (ljSSc) (p=.197). The mean age at first non-Raynaud’s symptoms was 10.0 years in the djSSc and 11.2 years in the ljSSc (p=0.247). Mean disease duration at time of inclusion was 3.4 years in the djSSc and 2.4 years in the ljSSc group.Significant differences were found between the groups regarding mean modified Rodnan skin score, 18.2 in the djSSc vs 6.2 in the ljSSc (p=0.02); presence of Gottron´s papulae (djSSc 30% vs ljSSc 13%, p=0.43);presence of teleangiectasia (djSSc 42% vs 18% ljSS, p=0.01); history of ulceration (djSSc 42% vs 18% ljSSc,p=0.008); 6 Minute walk test below the 10thpercentile (djSSc 85% vs ljSSc 54%, p=0.044), total pulmonary involvement (djSSc 49% vs ljSSc 31%, p=0.045), cardiac involvement (ljSSc 17% vs djSSc 3%, p=0.002). djSSc patients had significantly worse scores for Physician Global Assessment of disease activity compared to ljSSc patients (VAS 0-100) (40 vs 15) (p=0.001) and for Physician Global Assessment of disease damage (VAS 0-100) (36 vs 17) (p=0.001).There were no statistically significant differences in the other presentations. Pulmonary hypertension occurred in approximately 6% in both groups. No systemic hypertension or renal crisis was reported. ANA positivity was 90% in both groups. Anti-Scl70 was positive in 35% in djSSc and 36% in the ljSSc group. Anticentromere positivity occurred in 3% in the djSSc and 7% in the ljSSc group.Conclusion:In this unique large cohort of jSSc patients there were significant differences between djSSc and ljSSc patients at time of inclusion into the cohort regarding skin, vascular, pulmonary and cardiac involvement. According to the physician global scores the djSSc patients had a significantly more severe disease. Interestingly the antibody profile was similar in both scleroderma phenotypes.Supported by the “Joachim Herz Stiftung”Disclosure of Interests: :Ivan Foeldvari Consultant of: Novartis, Jens Klotsche: None declared, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Maria T. Terreri: None declared, Ana Paula Sakamoto: None declared, Valda Stanevicha: None declared, Flávio R. Sztajnbok: None declared, Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Brian Feldman Consultant of: DSMB for Pfizer, OPTUM and AB2-Bio, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Maria Katsikas: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, edoardo marrani: None declared, Mikhail Kostik: None declared, Natalia Vasquez-Canizares: None declared, Simone Appenzeller: None declared, Mahesh Janarthanan: None declared, Monika Moll: None declared, Dana Nemcova: None declared, Anjali Patwardhan: None declared, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Sujata Sawhney: None declared, Dieneke Schonenberg: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Blanca Bica: None declared, Juergen Brunner Grant/research support from: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Abbvie, Roche, BMS, Speakers bureau: Pfizer, Novartis, Abbvie, Roche, BMS, Patricia Costa Reis: None declared, Despina Eleftheriou: None declared, Liora Harel: 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, Daniela Kaiser: None declared, Dragana Lazarevic: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Susan Nielsen: None declared, Farzana Nuruzzaman: None declared, Yosef Uziel: None declared, Nicola Helmus: None declared

Background:Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of around 3 in 1, 000,000 children. It is known that in pediatric jSSc cohorts, there are a significant number of patients with overlap features, such as arthritis and myositis. However, the disease burden between those with and without overlap features in jSSc has not been defined.Objectives:Compare the clinical phenotype between children with and without overlap features in the juvenile systemic scleroderma inception cohort (jSScC).Methods:A cross-sectional study was performed using baseline visit data. Demographic, organ system evaluation, autoantibody profile, treatment, and patient and physician reported outcome variables were extracted from jSScC. Comparison between patients with and without overlap features was performed using chi-square test and Mann Whitney U-test.Results:At the time of data extraction, 175 jSSc patients were enrolled in the cohort, 81% were Caucasian and 81% female. Mean disease duration was 3.1 year (±2.7). Mean age at Raynaud´s onset was 10 years (±3.8) and mean age of first non-Raynaud´s was 10.2 years (±3.8). Overlap features occurred 17% (n=30) of the cohort, 12.5% in the diffuse cutaneous (dc) jSSc and in 30% in the limited cutaneous (lc) jSSc. Significant differences in clinical characteristics were found between those patients with compared to without overlap characteristics. Patients with overlap features presented more frequently with Gottron papules (p=0.007), swollen joints (p=0.019), muscle weakness (p=0.003), and lung involvement documented by decreased DLCO < 80% (p=0.06) and/or abnormal high resolution computed tomography (p=0.049). Anti-PM/Scl autoantibodies were also more common in this group (p=0.001). Significantly more patients without overlap features had Raynaud´s (p=0.006). Physician Global Assessment of disease activity was significantly higher in patients with overlap features (41 vs 34; p=0.041). (Table 1.)Table 1.Demographic and clinical characteristics of jSSc patients with and without overlap features.Whole CohortN=175Patients without overlapN=145Patients with overlapN=30P valueFemale to Male Ratio 4.3:1(142/33)4:1(116/29)6.5:1(26/4)0.395Cutaneous subtypeDiffuse subtype (N)73% (128)11216Limited subtype (N)27% (47)3317Mean disease duration (years)3.1 (± 2.7)3.2 (± 2.8)3.1 (± 2.2)0.291Mean age of onset of Raynaud´s (years)10.0 (± 3.8)17 non-Raynaud10.0 (± 3.8)10 non-Raynaud10.0 (± 3.7)7 non-Raynaud0.931Mean age of onset of non-Raynaud´s (years)10.2 (± 3.8)10.2 (± 3.9)9.8 (± 3.7)Disease modifying drugs (N)88% (154) 89% (129)83% (25)0.388Raynaud´s phenomenon90% (158)93% (135)77% (23)0.006Anti-PMScl18% (12/68)9% (5/53)47% (7/15)0.001Gottron Papules (N)27% (46/171)23% (33/144)48% (13/27)0.007DLCO 44% (39/88)39% (28/71)65% (11/17)0.06Abnormal findings in HRCT (N)44% (59/133)40% (43/107)62% (16/26)0.049Proportion of patients with swollen joints 18% (32) 14% (21) 37% (11)0.019Muscle Weakness (N) 21% (31/149)16% (20/123) 42% (11/26)0.003Physician global disease activity(0-100) min -max35 (0-90) n=14134 (0-90) n=11441 (0-80) n=270.041Conclusion:Results from this large international cohort of jSSc patients demonstrate significant differences between patients with and without overlap features. Patients with overlap have significantly more interstitial lung disease and more physician rated disease activity and should not be considered to have more “mild disease”.Supported by the “Joachim Herz Stiftung”Disclosure of Interests:None declared

Background:Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of jSSc the juvenile systemic sclerosis inception cohort (jSScC) has been prospectively enrolling patients with predetermined clinical variables over the past 12 years. One of the goals is to study the demographic, clinical features, and physician and patient reported outcome differences between those with juvenile limited cutaneous (lc) compared to diffuse cutaneous (dc) disease subtypes, to determine if characteristics are similar or different between dc and lc jSSc.Objectives:Evaluation of the baseline clinical characteristics of jSSc patients in the jSScC. Compare clinical phenotype between diffuse (dcjSSc) and limited cutaneous (lcjSSc) subtypes.Methods:Demographic, physical examination, organ system evaluation, autoantibody profile, treatment, and patient and physician reported outcome variables were evaluated from the jSSc Inception cohort and summary statistics applied using chi-square test and Mann Whitney U-test comparing lcjSSc and dcjSSc subtypes.Results:At the time of data extraction, 175 jSSc patients were enrolled in the cohort, 81% were Caucasian and 81% female. Diffuse cutaneous jSSc subtype predominated (73%). Mean disease duration was 3.1 year (±2.7). Mean age at Raynaud´s was 10 years (+3.8) and mean age of first non-Raynaud´s was 10.2 years (±3.8). Significant differences were found between dcjSSc versus lcjSSc, regarding several clinical characteristics. Patients with diffuse cutaneous subtype had significantly higher modified Rodnan skin score (p=0.001), presence of sclerodactyly (p=0.02), presence of Gottron’s papules (p=0.003), presence of telangiectasia (p=0.001), history of digital tip ulceration (p=0.01), and frequency of elevated CK value (p=0.04). Cardiac involvement was significantly higher in limited cutaneous jSSc subtype (p=0.02). Diffuse cutaneous jSSc patients had significantly worse scores for Physician Global Assessment of disease activity (38 vs 25; p=0.002) and disease damage (34 vs 19; p=0.008).Table 1.Comparison of demographic data and significant differences between dcjSSc and lcjSSc at time of inclusionWhole CohortN=175Diffuse SubtypeN=128Limited SubtypeN=47P valueFemale to Male Ratio4.3:1 (142/33)4.1:1 (103/25)4.8:1 (39/8)0.829Cutaneous subtypeDiffuse subtype73% (128)1280Limited subtype27% (47)047Mean Disease duration (years)3.1 (± 2.7)3.3 (± 2.9)2.6 (± 2.2)0.135Mean age of onset of Raynaud´s (years)10.0 (± 3.8)17 non-Raynaud9.8 (± 3.6)10 non-Raynaud10.6 (± 4.3)7 non-Raynaud0.219Mean age of onset of non-Raynaud´s (years)10.2 (± 3.9)10.0 (± 3.7)10.9 (± 4.3)0.173Disease modifying drugs88% (154)89% (114)85% (40)0.446CutaneousMean modified Rodnan skin score14.3 (0-51)17.4 (0-51)6.1 (0-24)0.001Gottron Papules27% (46/171)33% (41/124)11% (5)0.003Sclerodactyly78% (126/162)82% (98/119)65% (28/43)0.020Laboratory valuesElevated CK25% (30/122)30% (26/88)12% (4/34)0.041VascularTelangiectasia36% (56/154)44% (49/111)16% (7/43)0.001History of ulceration53% (91/173)61% (77/127)30% (14/46)0.001CardiacCardiac Involvement6% (10)2% (3)15% (7)0.002Patient Related OutcomesPhysician global disease activity(0-100) min -max35(0-90) n=14138(0-90) n=10825(0-80) n=330.002Physician global disease damage(0-100) min -max31(0-85) n=14034(0-85) n=10819(0-60) n=320.008Conclusion:Results from this large international cohort of jSSc patients demonstrate significant differences between dcjSSc and lcjSSc patients. According to the general organ involvement and physician global scores, the dcjSSc patients had significantly more severe disease. These observations strengthen our previous findings of the unique organ pattern of pediatric patients.Supported by the “Joachim Herz Stiftung”Disclosure of Interests:None declared.

Background:The Composite Response Index in Systemic Sclerosis (CRISS) was developed by Dinesh Khanna as a response measure in patients with adult systemic sclerosis. CRISS aims to capture the complexity of systemic sclerosis and to provide a sensitive measure for change in disease activity. The CRISS score is based on a two-step approach. First, significant disease worsening or new-onset organ damage is defined as non-responsiveness. In patients who did not fulfill the criteria of part one, a probability of improvement is calculated for each patient based the Rodnan Skin Score (mRSS), percent predicted forced vital capacity (FVC%), patient and physician global assessments (PGA), and the Health Assessment Questionnaire Disability Index (HAQ-DI). A probability of 0.6 or higher indicates improvement.Objectives:The objective of this study was to validate the CRISS in a prospectively followed cohort of patients with juvenile systemic sclerosis (jSSc).Methods:Data from the prospective international inception cohort for jSSc was used to validate the CRISS. Patients with an available 12-months follow-up were included in the analyses. Clinically improvement was defined by the anchor question about improvement (much better or little better versus almost the same, little worse or much worse) in patients overall health due to scleroderma since the last visit provided by the treating physician.Results:Forty seven jSSc patients were included in the analysis. 74.2% had diffuse subtype. The physician rated the disease as improved in 34 patients (72.3%) since the last visit. No patient had a renal crisis or new onset of left ventricular failure during the 12-months follow-up. Three patients (3.4%) each had a new onset or worsening of lung fibrosis and new onset of pulmonary arterial hypertension. In total, 6 patients resulted in a rating of not improved based on the CRISS in part I. The mRSSS, FVC%, CHAQ and PGA significantly improved during the 12-months follow-up in patients who were rated as improved. The predicted probability based on the CRISS algorithm resulted in an area under curve of 0.77 predicting the anchor question of improvement. In summary, 33 (70.0%) patients were correctly classified by the adult CRISS score resulting in an overall area under curve of 0.7.Conclusion:The CRISS score was evaluated in a pediatric jSSc cohort for the first time. It showed a good performance. However, it seems that the formula of part II of the CRISS score needs a calibration to pediatric jSSc patients.Disclosure of Interests:Jens Klotsche: None declared, Ivan Foeldvari Consultant of: Novartis, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Valda Stanevicha: None declared, Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, edoardo marrani: None declared, Maria T. Terreri: None declared, Flávio R. Sztajnbok: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Despina Eleftheriou: 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, Farzana Nuruzzaman: None declared, Nicola Helmus: None declared

Background:Familial Mediterranean Fever (FMF) is the most common periodic fever syndrome in childhood with an autosomal recessive inheritance pattern and is characterized by unprovoked fever attacks, serositis episodes. The causative gene of the disease is MEFV that encodes pyrin protein. The pyrin protein takes a role in pathways related to inflammation, and mutations of it lead to increased inflammation. It is already shown that frequencies of some certain diseases like PAN, HSP increase in patients with FMF. Nevertheless, frequencies of inflammatory diseases in families of patient with FMF haven’t been investigated.Objectives:In this study, we have aimed to evaluate the comorbid disorders in a large cohort of families of patients with FMF.Methods:Four hundred and ninety-eight children with FMF, one hundred and forty patients with JIA and ninety-two healthy children were interviewed between December 2019 and January 2020. In JIA group and healthy control group, patients who have family history for FMF were excluded from the study. Patients were asked about characteristics of their disease attacks and if there is a relative with any inflammatory diseases who does not have FMF in patient’s 1stand 2th degree relatives.Results:Demographic features of study group have shown in Table 1. The most common MEFV mutations in patients with FMF were: M694V homozygotes (13.2 %), M694V heterozygotes (12 %), M694V homozygotes and R202Q homozygotes (6,8 %). Type II diabetes, asthma and hypothyroidism were the most commonly detected diseases in all cohorts. Frequency of Behçet’s disease, allergic rhinitis and type II diabetes were significantly higher in families of patients with FMF than other groups (pTable 1.Demographic features of study population.FMF†JIA††Healthy Controln: 498 (%)mean +/- SDn: 140 (%)mean +/- SDn: 92 (%)mean +/- SDFemale284 (57)91 (65)55 (59.8)Age (years)12.9 ± 8.211.7 ± 5.17.4 ± 4.6Age at Onset (years)4.3 ± 3.35.4 ± 4.1-Age at Diagnosis (years)6.3 ± 3.66.3 ± 4.5-Delay in Diagnosis (months)23.8 ± 29.211.3 ± 28.2-Follow-up Duration (years)6.9 ± 8.35.3 ± 4.0-Consanguinity100 (20)25 (17.8)8 (8.6)Family History of FMF282 (56.6)0 (0)0 (0)JIA subgroup-- Oligoarticular72 (51.4) Polyarticular (RF negative)16 (11.4) Polyarticular (RF positive)3 (2.1) Enthesitis Related Arthritis14 (10) Psoriatic Arthritis7 (5) Systemic23 (16.4) Other5 (3.5)Clinical Findings-- Fever392 (78.1) Abdominal Pain429 (86.1) Chest Pain102 (20.5) Arthralgia334 (67.1) Arthritis157 (31.5) Extremity Pain64 (12.8) Heel Pain44 (8.8) Myalgia43 (8.6) *ELE13(2.6) Serositis10 (2)†Familial Mediterranean Fever††Juvenile Idiopathic Arthritis*Erysipelas like erythemaTable 2.Comparison of frequencies of diseases detected among families of patient groups (shortened).DiseasesFMFJIAHealthy Controlp1Type II Diabetes284 (57)64(45.7)44 (47.8)0.02Asthma139 (27.9)30 (21.4)20 (21.7)0.19Hypothyroidism122 (24.4)27 (19.2)14 (15.2)0.09Eczema68 (13.6)14 (10)5 (5.4)0.06Psoriasis49 (9.8)6 (4.2)7 (7.6)0.10Allergic Rhinitis49 (9.8)3 (2.1)1 (1)0.001Hyperthyroidism40 (8)9 (6.4)3 (3.2)0.24Behçet’s Disease31 (6.2)1 (1)2 (2.1)0.01Rheumatic Fever30 (6)10 (7.1)2 (2.1)0.25Conclusion:In this study, we have reported increased frequencies of Behçet’s disease, allergic rhinitis and type II diabetes in families of patients with FMF. Our results suggest that possible increased mutation load among families of patients with FMF may cause increased inflammatory diseases.References:[1]Yildiz M, Adrovic A, Tasdemir E, et al. Evaluation of co-existing diseases in children with familial Mediterranean fever.Rheumatol Int. 2020;40(1):57–64. doi:10.1007/s00296-019-04391-9Disclosure of Interests:None declared

Background:Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence in around 3 in a million children. Pulmonary involvement in jSSc occurs in approximately 40 % in the inception cohort. Traditionally in jSSc, pulmonary function testing (PFT) with FVC and DLCO are used for screening and computed tomography (HRCT) was more reserved for those with abnormal PFTs. More recently, it has become apparent that PFTs might not be sensitive enough for detecting ILD in children.Objectives:Utilizing a prospective international juvenile systemic scleroderma cohort (JSScC) [2], to determine if pulmonary screening with FVC and DLCO is sufficient enough to assess the presence of interstitial lung disease in comparison to CT evaluation.Methods:The international juvenile systemic scleroderma cohort database was queried for available patients with recorded PFT parameters and HRCT performed to determine sensitivity of PFTs detecting disease process.Results:Of 129 patients in the jSScC, 67 patients had both CT imaging and an FVC reading from PFTs for direct comparison. DLCO readings were also captured but not in as many patients with tandem HRCT (n =55 DCLO and HRCT scan). Therefore, initial analyses focused on the sensitivity, specificity and accuracy of the FVC value from the PFTs to capture the diagnosis of interstitial lung disease as determined by HRCT.Overall, 49% of the patients had ILD determined by HRCT, with 60% of patients having normal FVC (>80%) with positive HRCT findings, and 24% of patients having normal DLCO (> 80%) with positive HRCT findings. Fourteen percent (n = 3/21) of patients with both FVC and DLCO values within the normal range had a positive HRCT finding.Conclusion:The sensitivity of the FVC in the JSScC cohort in detecting ILD was only 39%. Relying on PFTs alone for screening for ILD in juvenile systemic sclerosis would have missed the detection of ILD in almost 2/3 of the sample cohort, supporting the use of HRCT for detection of ILD in children with SSc. In addition, the cut off utilized, of less than 80% of predicted FVC or DLCO could be too low for pediatric patients to exclude beginning ILD. This pilot data needs confirmation in a larger patient population.Supported by the “Joachim Herz Stiftung”Disclosure of Interests:Ivan Foeldvari Consultant of: Novartis, Bernd Hinrichs: None declared, Kathryn Torok: None declared, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Valda Stanevicha: None declared, Flávio R. Sztajnbok: None declared, Maria T. Terreri: None declared, Ana Paula Sakamoto: None declared, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Maria Katsikas: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, Rolando Cimaz: None declared, Mikhail Kostik: None declared, Simone Appenzeller: None declared, Mahesh Janarthanan: None declared, Monika Moll: None declared, Dana Nemcova: None declared, Dieneke Schonenberg: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Blanca Bica: None declared, Juergen Brunner Grant/research support from: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Abbvie, Roche, BMS, Speakers bureau: Pfizer, Novartis, Abbvie, Roche, BMS, Patricia Costa Reis: None declared, Despina Eleftheriou: None declared, Liora Harel: 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, Dragana Lazarevic: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Susan Nielsen: None declared, Farzana Nuruzzaman: None declared, Anjali Patwardhan: None declared, Yosef Uziel: None declared, Nicola Helmus: None declared

Background:Childhood-onset Takayasu Arteritis (c-TAK) may differ from adult-onset Takayasu Arteritis (a-TAK) in clinical maaifestations and treatment.Objectives:To compare c-TAK with a-TAK patients for vascular involvement, disease activity, damage, and treatment.Methods:Patient charts from two tertiary-care centers of a pediatric and adult clinic were reviewed. Adult patients diagnosed before the age of 18 were included in the c-TAK group. The activity was assessed with the physician’s global assessment (PGA) and Indian Takayasu Clinical Activity Score (ITAS). The damage was evaluated with Takayasu Arteritis Damage Score (TADS) and Vasculitis Damage Index (VDI).Results:Twenty four c-TAK and 121 a-TAK patients were compared. 21 (88%) of the c-TAK group and 104 (89%) of the a-TAK group were female. Age at symptom onset was 14 (IQR: 9-15) for c-TAK and 30 (IQR: 24-43) for a-TAK patients. Diagnostic delay in months was shorter for c-TAK patients [c-TAK: 3 (1-10) vs. a-TAK: 12 (5-58)]. Follow-up duration was similar [53 months (IQR: 16-131) vs. 68 (IQR: 30-102), p=0.763].ITAS was comparable for c-TAK and a-TAK patients on the first visit [14 (SD: 7) vs. 13 (SD: 5), p=0.362, respectively]. However, the PGA score was higher in the c-TAK group compared to the a-TAK group [9 (IQR 7-10) vs. 7 (IQR 6-8), p14 (64%) of c-TAK patients and 10 (9%) of a-TAK patients received pulse glucocorticoids, p= 0.002. Cumulative glucocorticoid dose was 10 grams (IQR: 6-13) for c-TAK patients and 7 grams (IQR: 4-12) for a-TAK patients (p=0.128).After diagnosis, children had more vascular interventions than the adults did [9 (38%) vs. 20 (18%), p=0.031, respectively].Rates of achieving at least one remission were lower for c-TAK patients [c-TAK: 12 (50 %) vs. a-TAK: 94 (82%), p=0.001]. c-TAK patients had a PGA score of 6 (IQR 3-8), the PGA score in a-TAK patients was 1 (IQR 1-3), pTADS was similar [c-TAK: 8 (IQR 4-12), a-TAK: 8 (IQR 6-10), p=0.919]. However, VDI of the a-TAK patients was higher than the c-TAK patients [c-TAK: 4 (IQR 2-5), a-TAK: 5 (IQR 3-7), p=0.017]. Glucocorticoid related damage was higher in a-TAK patients (Diabetes: 8% vs. 4%, avascular necrosis: 6% vs. 0, and cataracts: 11% vs. 0)Conclusion:Aorta involvement, biologic agent use, and vascular interventions were more common in c-TAK patients. However, cumulative damage was not increased for c-TAK patients which may be partly explained by more common corticosteroid related side-effects in adults.Table 1.Baseline symptoms, physical examination findings*c-TAK (n= 24)a-TAK (n= 117)pSYMPTOMSStroke1 (4)8 (7)1Carotidynia019 (16)0.044Upper Extremity Claudication5 (21)72 (62)Hypertension13 (54)22 (19)Pulse loss (Radial)8/23 (35)62 (58)0.043BRUITSubclavian8 (35)62 (57)0.054Renal9 (39)15 (14)0.014Abdominal Aorta11 (48)9 (8)*Values denote the number (%) of patientsFigure 1.Comparison of involved arteries* * Numbers in bars represent percentage of patients in each groupFigure 2.Angiographic classification types according to Hata* * Numbers in bars represent percentage of patients in each group. Type 2a and Type 2b are combined. a-TAK group had no patient with Type 3 diseaseTable 2.Medical treatment*First TreatmentTreated Everc-TAK (n=22)a-TAK (n=115)pc-TAK(n=24)a-TAK (n=114)pMethotrexate5 (23)69 (60)0.00112 (50)76 (67)0.123Azathioprine8 (36)38 (33)0.76321 (88)79 (69)0.070Leflunomide01 (1)13 (13)35 (31)0.070Cyclophosphamide†6 (27)6 (5)0.00412 (50)10 (9)Biologics10 (42)16 (14)0.004Anti-TNF---4 (17)18 (16)-Tocilizumab---7 (29)4 (4)-*Values denote the number (%) of patients.Disclosure of Interests:None declared

Background:Pediatric to adult rheumatology transition can be a challenge for both the patient and the clinician, especially in rheumatology as it includes chronic diseases with close follow-up.Objectives:The objective of this study is to understand our tertiary rheumatology center patient demographic transitioning from pediatric to adult rheumatology in order to design prospective studies enhancing the evidence of transition recommendations.Methods:Patients included in this study are regularly followed-up in our adult rheumatology clinic and were regularly followed up in our pediatric rheumatology clinic in the past. They were all diagnosed with a rheumatologic condition receiving treatment. The patient files were assessed to have a better understanding of their demographic, disease and treatment information.Results:Our cohort includes 347 patients diagnosed with a variety of conditions that are Familial Mediterranean Fever (FMF) (n=216), Juvenile Idiopathic Arthritis (JIA) (n=56), Juvenile Spondyloarthritis (jSPA) (n=39), Systemic Lupus Erythematosus (SLE) (n=20), Behçet’s Disease (n=7) and the rest of the rheumatologic conditions with less than 5 patients each. The mean age of the patients during transition, mean age of diagnosis, and follow-up duration are 21.34±1.7, 10.4±4.18, and 10.82±4.4 in respective order. The treatment regimens the patients received are summarized in Table 1.Table 1.Current Treatment Information of the PatientsCurrent Treatment InformationDMARD26Colchicine23Adalimumab21Etanercept10NSAID4Tocilizumab3Cyclophosphamide3Rituximab2Prednisolone7Mycophenolate Mofetil1Canakinumab1Seven patients had FMF related attacks. In addition to attacks, one FMF patient had bilateral ankle pain and one patient had leg pain. One patient out of three diagnosed with Takayasu’s disease was still symptomatic. One patient had uveitis-related symptoms. One patient diagnosed with SLE had skin dryness. Furthermore, there were patients with sequelae formation. One patient diagnosed with oligoarticular JIA (oJIA) had bilateral hip sequela with the additional left hip prosthesis. One oJIA patient had micrognathia, and one had left knee sequela. One pJIA patient had small joint sequelae. One sJIA patient had bilateral hip sequelae. One jSPA patient had enthesopathy. One FMF patient had proteinuria due to amyloidosis formation. Another FMF patient had hip surgery due to sequela.Conclusion:Our center had patients with a variety of conditions with different natures of diseases. EULAR recommends the transition process to start no later than 14 years of age; however, this process started at the mean age of 21 in our patients. In most of these patients, especially the ones diagnosed with FMF, the control of disease activity was maintained. The transition of these different clinical entities might require certain amendments to the standard of care. For future references, we will be able to understand more about the adulthood prognosis of these clinical entities.Disclosure of Interests:None declared

Background:Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There is rare longitudinal prospective follow up data of patients with jSSc. In the international juvenile systemic scleroderma cohort (JSScC) patients are followed with a standardized assessment prospectively.Objectives:To assess the changes regarding organ involvement pattern and patients related outcomes after 36 months follow up in the JSScC.Methods:Patients diagnosed according the ACR 2013 criteria for systemic sclerosis were included, if they developed the first non-Raynaud symptom before the age of 16 and were under the age of 18 at the time of inclusion. Patients were followed prospectively every 6 months with a standardized assessment.Results:39 patients in the JSScC had 36 months follow up. 80% had a diffuse subtype. 95% of the patients were Caucasian origin. 31 of the patients were female (80%). Mean disease duration at time of inclusion was 3.5 years. Mean age onset of Raynaud’s was 8.8 years and mean age of onset at the first non-Raynaud´s was 9.5 years. Around 30% of the patients were anti-Scl70 positive and none of them anti-centromere positive. The MRSS dropped from the time point of the inclusion into the cohort from 13.9 to 11.8 after 36 months. Pattern of organ involvement did not show any significant change, beside the increase of the nailfold capillary changes from 49% to 73% (p=0.037). No renal crisis occurred. No mortality was observed.They were positive significant changes in the patient related outcomes. The physician global disease activity decreased from 40.0 to 22.1 assessed on a VAS scale of 0 to 100 (p Patients global disease activity decreased from 43.3 to 20.4 and patients global disease damage from 45.0 to 21.7 both assessed on a VAS scale of 0 to 100 (pConclusion:After 36 months follow up, we could observe a significant improvement of patient related outcomes and only one significant change in organ pattern involvement. In a mostly diffuse subset patient population this is a very promising result regarding outcome.Supported by the “Joachim Herz Stiftung”Disclosure of Interests:Ivan Foeldvari Consultant of: Novartis, Jens Klotsche: None declared, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Maria T. Terreri: None declared, Rolando Cimaz: None declared, Maria Katsikas: None declared, Dana Nemcova: None declared, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Juergen Brunner Grant/research support from: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Abbvie, Roche, BMS, Speakers bureau: Pfizer, Novartis, Abbvie, Roche, BMS, Mikhail Kostik: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Anjali Patwardhan: None declared, Kathryn Torok: None declared, Nicola Helmus: None declared

Background:Anti-nuclear antibodies (ANA) are a group of the antibodies that develop against intracellular components of the cells. It is usually useful for diagnosing some of the connective tissue diseases like systemic lupus erythematosus, mixed connective tissue disease. But it is reported that its positivity rate is about %20 in healthy individuals. Therefore, it can be confusing to check ANA test, if there is not really high suspicion for connective tissue diseases or juvenile idiopathic arthritis.Objectives:We aimed to evaluate results of long-term follow-up of the patients with ANA positivity who had initially no identifiable rheumatic diseases.Methods:Six hundred and ninety-four patients with ANA positivity who did not diagnosed as any of the rheumatic diseases at the first examination were found in database. Two hundred and eighty- two patients of them were called so far and questioned about their demographic features and symptoms that are related with rheumatic diseases.Results:Mean age of the patients at the time of study and at the time of testing were 13.4± 4.5 and 9.1±4.0 years. The female: male ratio was 1.05. Mean follow-up duration was 4.3±2.8 years. Most common reasons for the request for ANA test were arthralgia and skin eruptions. ANA testing was most commonly requested by a general pediatrists. Demographic features of the patients were summarized in Table 1.Table 1.Demographic features of the patients.n (%)Age (years)13.4± 4.5Female145 (51.4)Age at the time of testing (years)9.1±4.0Follow-up Duration (years)4.3±2.8Reason for testing Arthralgia99 (44.1)Skin Eruption54 (24.1)Check-Up20 (8.9)Arthritis13 (5.8)Gait abnormalities7 (3.1)Hair Loss6 (2.6)Fever5 (2.2)Uveitis2 (0.8)Recurrent abdominal pain2 (0.8)Who suggested testing?Pediatrician196 (87.5)Parents13 (5.8)Dermatologist7 (3.1)Ophthalmologist3 (1.3)Rheumatologist2 (0.8)Other3 (1.3)Positivity of acute phase reactants15 (5.3)History of infection before testing56 (24.3)History of drug-using before testing39 (17)Most of the diseases were diagnosed in patients with ANA positivity were not related with autoimmune mechanisms that associated with ANA positivity therefore, these diseases are thought to be coincidence. Only in 1 patients, systemic lupus erythematosus that has certain association with ANA positivity were diagnosed. All diseases that are diagnosed were shown in Table 2.Table 2.All diseases that are diagnosed in patients during the follow-up period.n (%)Hypermobility Syndrome29 (10.2)Urticaria7 (2.4)Hypothyroidism6 (2.1)Transient synovitis4 (1.4)Chronic ITP*4 (1.4)Scoliosis4 (1.4)Familial Mediterranean Fever3 (1)Cryopyrin associated periodic Syndrome2 (0.7)PFAPA syndrome**2 (0.7)Celiac Disease2 (0.7)Acute Rheumatic Fever2 (0.7)Fibromyalgia1 (0.3)Bone Tumor1 (0.3)Juvenile Idiopathic Arthritis1 (0.3)Henoch-Shöenlein Purpura1 (0.3)Myastenia Graves1 (0.3)Sever Disease1 (0.3)Vitiligo1 (0.3)Systemic Lupus Erythematosus1 (0.3)*Idiopathic Thrombocytopenic Purpura, **Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome.Conclusion:We are reporting that in only 0.3% of patients with ANA positivity who don’t have any diseases diagnosed initially, were diagnosed as rheumatologic diseases during to the follow-up period. Since positivity of ANA is also common in the healthy population, requesting this test in only patients with high suspicion for connective tissue disease will reduce confusion in terms of diagnosis.References:[1]Kasapcopur O, Ozbakir F, Arisoy N, Ingol H, Yazici H, Ozdogan H. Frequency of antinuclear antibodies and rheumatoid factor in healthy Turkish children. Turk J Pediatr 1999;41:67-71.Disclosure of Interests:None declared

Background Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between male and female patients with Systemic Sclerosis. There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (www.juvenilescleroderma.com) is a prospective standardized register for patients with jSSc. Objectives comparison of patients characteristic at the time of inclusion in the registry who are male or female. Methods Patients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compered the demographics and clinical characteristics of the male and female patients. Results Up till now 74 patients were enrolled, 54 with djSSc (76%) and 18 with ljSSc (24%). 14 (19%) of the patients were male (M) and 60 female (F) (81%). The mean disease duration at the time of inclusion was 9.3 in M and 9.2 in F patients. 74.4% of the M and 76.7% of the F had diffuse subset. The mean age of the onset of Raynaud symptomatic was 9.3 in M and 9.2 years in the F patients and the non-Raynaud symptomatic with 9.1 in M and 9.9 in F patients. At the time of the inclusion the mean modified Rodnan Skin Score was 20 in M and 15.1 in F patients. Anti-Scl 70 positivity was found in 42.9% in M and 32.1% in F patients. Anticentromere positivity occurred in 16.7% in M and 3.3% in F patients (p=0.027). Capillary changes were present in 50% of the M and 60% of F patients, but 50% in M and F had already history of ulcerations, but 28.6% in M and 15.5% in F had active ulceration. 57.1% of the M and 50% of the F patients had cardiopulmonary involvement. Six patients had pulmonary hypertension, they were all F. 75% of M and 46.7% of F patients had signs of interstitial lung disease on imaging. Renal involvement was around 7% in both sexes. 21.4% in M and 38.3% in F patients had gastrointestinal involvement. 92.9% of M and 55.9% in M patients had musculoskeletal involvement. Conclusions We present the data on the first 74 patients with jSSc included in our cohort. Patients with male sex have a more severe disease similar to adult male patients. Disclosure of Interest None declared

Background Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc. Objectives comparison of patients characteristic at the time of inclusion with ljSSc and djSSc Methods We compered the demographics and clinical characteristics of the ljSSc and djSSc. Results Up till now 74 patients were enrolled, 54 (76%) with djSSc and 18 with ljSSc (24%). 9% in djSSc and 25% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.3 years in ljSSc. 82% in the djSSc and 78% in the ljSSc group were female. The mean age of the onset of Raynaud symptomatic was 9.0 years in the jdSSc and 9.9 years in ljSSc group and onset of the non-Raynaud symptomatic with 9.4 in djSSc and 10.6 ljSSc. At the time of inclusion the mean modified Rodnan Skin Score was 18.5 in the djSSc and 8,4 in ljSSc (p=0.0001). Anti-Scl 70 positivity was found in 31.5% of djSSc and 30.8% in ljSSc. Only 2 patient in the djSSc group and one in the ljSSc group was anticentromere positive. Capillary changes occurred in 60.7% in the djSSc and 50% in ljSSc, but 58.2% in djSSc and only 23.5% in ljSSc had already history of ulcerations (p=0.013) and 21.8% had active ulceration in the djSSc and 5.9% in the ljSSc. 33.3% of djSSc and 72.7% of ljSSc had cardiac involvement (p=0.027). pulmonary hypertension occurred in 57.1% djSSc and 18.2% in ljSSc. 63% in djSSc and 27.3% in ljSSc group had signs of interstitial lung disease on imaging (p=0.046). Renal involvement occurred in 7.1% djSSc and 5.6% in ljSSc. 39.3% of djSSc and 22.2% of ljSSc had gastrointestinal involvement. 56.4% in djSSc and 83.35 in ljSSc had musculoskeletal involvement (p=0.04). Conclusions Patients with djSSc have younger age at onset, have more often capillary changes and active ulcerations, pulmonary hypertension and less gastorintestinal and joint involvement and more disease damage. The characteristics of the pediatric subtypes differs from adults with SSc, especially the high proportion of patients with diffuse subtype. Disclosure of Interest None declared

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small-vessel vasculitis most commonly presents in adults with the average age of diagnosis is being 50 years. After the long-standing prodromal phase (asthma, allergic rhinitis, nasal polyposis) patients progress to eosinophilic phase which is marked by eosinophilia and pulmonary infiltrates and then to the vasculitic phase (1,2). Objectives Herein, we present a child in the vasculitic phase of EGPA with a severe skin involvement, who was initially diagnosed and treated for cutaneous anthrax. Results A 14-year-old male previously misdiagnosed as cutaneous anthrax was referred with a 2-month history of multiple wide and deep ulceronecrotic lesions in lower extremities, started after contact with animals (1a). Complete blood count (CBC) revealed anemia (hemoglobin: 8.5 g/dl, hematocrit: 26.9%), eosinophilia (4100/mm3) and thrombocytopenia (34000/mm3). Peripheral blood smear also revealed marked eosinophilia (42%). Infectious etiologies such as cutaneous anthrax, leishmaniosis, aspergillosis and nonspecific agents were excluded by gram staining, wound-blood-bone marrow cultures and serological investigations. Bone marrow aspiration was performed owing to the prominent eosinophilia, anemia and thrombocytopenia. However, the result was also pointing out eosinophilia without signs of eosinophilic leukemia. Although ANCAs were negative, the robust clinical manifestations concordant with EGPA (e.g. eosinophilia, increased Ig E level, history of asthma, vasculitic lesions) had led us to repeat the skin biopsy and search for lung involvement. Pulmonary high resolution computed tomography (HRCT) demonstrated bilateral ground glass opacity (figure 1c) and millimetric subpleural nodules (figure 1d). Skin biopsy revealed necrotizing inflammation without apparent granulomas and marked eosinophilic infiltrations in the extravascular areas. On the second day of hospitalization he developed deep vein thrombosis (DVT). A diagnosis of EGPA was established and intravenous methylprednisolone with low–molecular weight heparin were administered. Patient showed remarkable improvement of cutaneous lesions and DVT (Figure 1b). Conclusions Lack of awareness for EGPA among paediatricians as a consequence of limited childhood cases, results with diagnostic delay. Diagnosis of EGPA is challenging in the vasculitic phase and necessitates a detailed history that specifically questions the patient for asthma history. Coexistence of vasculitic lesions and eosinophilia associated with a prior history of asthma should lead to the consideration of EGPA in differential diagnosis. References Barut K, Sahin S, Kasapcopur O. Pediatric vasculitis. Curr Opin Rheum 2016;28:29–38. Gendelman S, Zeft A, Spalding SJ. Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort. J Rheumatol 2013;40:929–935. Disclosure of Interest None declared

Background Myocardial dysfunction due to coronary arterial lesions is an important complication after Kawasaki disease in childhood (1,2). Speckle-tracking echocardiography (STE) is a recently developed technique for evaluation of myocardial deformation (3). Objectives Our objective was to examine myocardial deformation at long term follow-up of Kawasaki patients. Methods We assessed regional myocardial function in 32 asymptomatic Kawasaki patients and 30 age matched healthy child as control group. STE examination were recorded and the acquired raw data were saved for offline analysis The inferoseptal, anterolateral, inferior, anterior, and inferolateral (posterior) walls were investigated for longitudinal systolic peak values for strain (LV-SR) imaging. Results Mean age was 21±12 months, mean follow up time 61 months, Left ventricular ejection fraction was 57,373±8,338 in patients and 61,100±3,804 in control group. LV-SR of Kawasaki patients at basal-inferoseptal (20,000±6,046 vs 25,200±5,074), basal-anterolateral (19,595±5,713 vs 22,733±3,494), apical-septal (23,378±5,609 vs 26,267±4,334), apical-inferior (24,054±3,696 vs 25,200±2,396) segments had lower values than control group. Conclusions We demonstrated subclinical systolic impairment of function especially at left ascending artery territories with STE. STE has the potential to detect these subtle abnormalities, and help management during long term follow up. References Printz BF, et al. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. Journal of the American College of Cardiology 2011;57(1): 86–92. Xu QQ, et al. Evaluation of left ventricular systolic strain in children with Kawasaki disease. Pediatric cardiology 2014;35(7): 1191–1197. Marwick TH, et al. Myocardial strain measurement with 2 dimensional speckle-tracking echocardiography: definition of normal range. JACC: Cardiovascular Imaging 2009;2(1): 80–84. Disclosure of Interest None declared

Background Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. According to organ involvement, the disease is divided into two main forms: systemic and localized. Localized scleroderma (JLS) is characterized with sclerosis of the skin but internal organs involvement is not expected. Juvenile systemic sclerosis (JSS) is characterized by both cutaneous and internal organ involvement. Clinical features are insidious and it can take years until complete clinical picture develops. Objectives The aim of the study was to investigate main demographic and clinical characteristics of patients with JS, followed up at our department. Additionally, we aim to share our experience of this rare condition. Methods Patients with JS were included in cross-sectional study. Demographic data were taken from disease history of patients. Clinical features, laboratory results and treatment options were evaluated at last clinical visit. Results A totally of 46 patients were included in the study: 40 (86,9%) female, 6 (13%) male. The mean age of patients was 14±3 years, mean age at disease onset was 9,1±4,23 years and the mean age at diagnosis was 10,2±3,4 years. Twenty six patients (56,5%) were diagnosed with JSS and 20 (43,4%) had JLS. Skin involvement and peripheral vasculopathy were the most common clinical features. Musculoskeletal involvement takes a second place. Gastro-intestinal (GIS) involvement was present only in systemic sclerosis group. Six patients (13%) had lung fibrosis. Pulmonary hypertension was found in 6,5% of all JS patients but its percentage was higher among patients with JSS (11,5%). None of patients had neurological and renal involvement. All JSS patients were positive while 40% of JLS patients were negative for ANA. Anti Scl 70 antibody was positive only among patients with JSS. Combination of MTX+corticosteroids was used in 15 patients (32,6%). Vasoactive agent was added in therapy of 20 patients (43,5%) which all belong to JSS group. While 31 patients were in remission (67,4%), 15 (32,6%) had active disease in last visit. All patients with active disease belonged to systemic group. Conclusions Juvenile scleroderma is rarely seen multisystemic disease. Unlike adults, cardiovascular and pulmonary involvement is uncommon among children. Early diagnosis, regular follow up and appropriate treatment are of high importance in clinical course and disease prognosis. References Zulian F, Cuffaro G, Sperotto F. Scleroderma in children: an update. Curr Opin Rheumatol 2013;25:643–50. Foeldvari I. New developments in juvenile systemic and localized scleroderma. Rheum Dis Clin N Am 2013;39: 905–20. Hedrich CM, Fiebig B, Hahn G, Suttorp M, Gahr M. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease. Clin Pediatr 2011;50: 604–14. Adrovic A, Oztunc F,Barut K, Koka A, Gojak R, Sahin S, et al. The frequency of pulmonary hypertension in patients with juvenile scleroderma. Bosn J Basic Med Sci. 2015;15:30–5. Disclosure of Interest None declared

Background Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by vasculitis and inflammation in various organs. Cardiovascular involvement, although not frequent in juvenile-onset SLE (j-SLE), if present, is a significant cause of morbidity and mortality (1). Particularly, involvement of the myocardium layer may lead to ventricular dysfunction since its progression is insidious (1,2). Speckle tracking echocardiography (STE) could demonstrate subclinical myocardial deformations (strain) that globally seems normal in conventional echocardiography (2). Objectives The aim of this study is early detection of subclinical systolic dysfunctions in j-SLE with STE and if present, then to investigate whether this is disease-related or it is a result of other predisposing conditions. Methods 35 patients with j-SLE and 30 healthy children as a control group were evaluated between January and August 2015 at outpatient clinics of Cerrahpasa Medical Faculty. STE was performed on all patients and controls. Medical records that are including age at diagnosis, duration of the disease, diagnostic criteria, laboratory tests and cumulative clinical manifestations were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). A SLEDAI score >4 was arbitrarily designated as a sign of moderate/severe disease activity. Results j-SLE patients had lower EF values than control subjects. Left ventricular end diastolic dimension (LVEDD) and left ventricular end systolic dimension (LVESD) were significantly greater in j-SLE patients (42,278±4,530 vs. 37,314±5,535; 28,108±3,344 vs. 24,055±3,290 p=0.001, respectively) than in the control group. There was a significant reduction in systolic parameters of longitudinal strain in the j-SLE group (p SLE patients were divided into two subgroups. Group 1 included patients having SLEDAI scores >8 at the beginning of the disease but who improved with therapy during follow up, with resulting SLEDAI scores less than or equal to 4 points. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis but with SLEDAI scores still greater than 4 at the end of follow up. In comparisons of two groups, mid inferior and mid inferolateral LV segment STE strain measurements of Group 2 were significantly lower than those in Group 1, (15.9000±6.47130 vs. 20.0714±4.49725 mid inferior; 17.9000±7.23341 vs. 23.2308±3.87629 mid inferolateral, p=0.075, 0.055, respectively). Conclusions We can say that prevention of long-term cardiovascular complications in j-SLE begins with noticing the iceberg early, before irreversible changes take place. In this regard, STE is an accurate method for detecting subclinical systolic dysfunction. References Apte M, McGwin G Jr, Vila LM, Kaslow RA, Alarcόn GS, Reveille JD. Associated factors and impact of myocarditis in patients with SLE from LUMINA, a multiethnic US cohort. Rheumatology (Oxford) 2008;47: 362–367. Huang BT, Yao HM, Huang H. Left ventricular remodeling and dysfunction in systemic lupus erythematosus: A three-dimensional speckle tracking study. Echocardiography 2014; 31: 1085–1094. Disclosure of Interest None declared

Background Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies assessment of the organ involvement is not standardized. Our project is the first one, where prospectively and with a standardized assessment data of jSSc patients are collected. Objectives to learn about the characteristics and evolvement of jSSc Methods Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol. Results 26 centers from 17 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 74 patients were enrolled. Sixty (81%) of the 74 patients were female. The mean age of the onset of Raynaud symptomatic was 9.2 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,7 years (0.3 -15.9). 56 (76%) of the 74 have diffuse subtype, 10 (14%) of them have an overlap symptomatic. At the time of the inclusion the mean modified Rodnan Skin Score was 16.0. ANA positive were 55/71 (77%), 24/70 (34%) of them were anti-Scl 70 positive and 3/42 (7%) was anticentromere positive. 43/74 (58%) had already capillary changes and 36/72 (50%) inactive ulcerations, 13/72 (18%) had active ulceration at the time of the inclusion. 38/74 (51%) had cardiopulmonary involvement, 19/38 (50%) of had signs of interstitial lung disease on imaging, 18/42 (43%) had FVC Conclusions The current recruitment data confirms that pediatric patients are different from the adult patients, there is a significantly higher proportion of diffuse subset patients with 81%. 14% of the patients have overlap features. Disclosure of Interest None declared

A 14-year-old boy previously misdiagnosed as having cutaneous anthrax was referred with a 2-month history of multiple wide and deep ulceronecrotic lesions in the lower extremities, which occurred after contact with animals. Skin biopsy was compatible with vasculitis. Further examination at our hospital elicited eosinophilia and a history of asthma. On the second day of hospitalisation, he developed deep vein thrombosis. A diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was established and intravenous methylprednisolone was administered. The patient showed remarkable improvement of the cutaneous lesions. Diagnosis of EGPA is challenging in the vasculitic phase and necessitates a detailed history that specifically questions the patient for an asthma background. This case illustrates a severe cutaneous presentation of EGPA and emphasises the difficulty of diagnosis as a result of overlapped signs and symptoms with cutaneous anthrax and leukaemia. EGPA should be kept in mind in the differential diagnosis of cutaneous lesions associated with eosinophilia.

Background Juvenile spondyloarthropathies (JSpA) are a group of chronic childhood rheumatic diseases which emerge especially before 16 and after 6 years-old. While enthesitis and oligoarthritis are the major signs of the early period, axial skeletal involvement and sacroiliitis are the late period signs. Although the diagnosis of the spondyloarthropathy is easy in adults, it is quite difficult in children. Objectives To determine clinical and demographical features of the JSpA cases that is followed-up in our department, and to evaluate treatment modalities and long term complications of disease together with the side effects of the drugs. Methods 107 children (21 female, 86 male) that admitted to our clinic with the diagnosis of JSpA between January 2005-December 2014 were involved in our study. Enthesitis related arthritis and Juvenile Ankylosing Spondylitis were included under the topic of JSpA. Clinical and laboratory variables were obtained from patient records. Results While the mean age of the disease onset and diagnosis were 11,4±3 years (range 3 -17 years) and 12,4±2,8 years (range 5-18 years) respectively, the mean duration of follow-up period was 2,7±2,5 years (range 2 months-12 years). Lower extremity arthritis (n=83, 77,6%), hip pain (n=63, 58,9%) and inflammatory low back pain (n=47, 43,9%) were the major clinical findings. The most seen involvement of the lower extremity was ankle joint arthritis (n=60, 72,3%) followed by enthesitis (n=76, 71%) and tarsitis (n=30, 28%). Axial skeletal involvement was noted in 57% (n=61) of patients and mean inflammatory low back pain duration was 8,3±6,9 months (range 1-24 months). Magnetic resonance imaging could be studied in 77 patients (72%) that imaging results of 52 children (48,6%) were consistent with sacroiliitis. Lumbar movement limitation in Schober9s test was seen in 42 cases (39.3%). Family history of spondylitis in 29 children (27.1%) and familial Mediterranean fever (FMF) in 4 cases. Furthermore, 5 patients diagnosed as FMF and treated with colchicine. Uveitis was seen in 11 children (10.3). Cardiological, pulmonary and renal investigations were normal of the whole study. HLA-B27 tissue antigen was positive in 73.8% (n=79) of the patients. First line therapies were methotrexate in 51 cases (47.5%) and sulphasalazine in 40 cases (37.4%). Anti-TNF alpha agents were used in 51 patients (47,7%) that were resistant to first line therapy. In the last medical visits we evaluated the patients in the terms of treatment modalities; 60.7% (n=65) of cases were noted as using drugs and in remission, 12.1% (n=13) gave up drugs and in remission, 16.8% (n=18) as minimally active and 6,5% (n=7) as active. Conclusions Initial signs of JSpA are usually lower extremity arthritis and enthesitis in children that is quite different from adults. JSpA should be strongly suspected in the case of a boy that is older than 6 years-old with lower extremity arthritis and the family history of spondyloarthropathy. Afterwards, axial skeletal evaluation must be investigated immediately and in order to prevent this complication true and efficient therapy must be started. References Tse SML, Laxer RM. New advances in juvenile spondyloarthritis. Nat Rev Rheumatol 2012;8:269-79 Kasapcopur O, Demirli N, Ozdogan H et al. Evaluation of classification criteria for Juvenile-onset spondyloarthropathies. Rheumatol Int. 2005;25:414–18 Disclosure of Interest None declared