Background Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children (1). There are limited data published regarding the differences in clinical presentation of male and female patients with jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is a multinational cohort with a prospective standardized assessment of the patients. The data regarding the difference in clinical characteristics at time of inclusion in the cohort are presented. Objectives Evaluation of the differences in clinical presentation of male and female jSSc patients at the time of inclusion in the JSSIC. Methods The JSSIC is a prospective multicentre registry of patients with jSSc, who fulfil the adult classification criteria (2), and presented the first non-Raynaud symptoms before 16 years old and were younger than 18 years old at the time of inclusion in the cohort. Patients characteristics at time of inclusion in the cohort were evaluated. Results As of 15th of December 2018 120 patients are included in JSSIC. The great majority are female (80%). There were more female patients with CK elevation (29% vs 22%) and more female patients with Gottron papulae (25% vs 12%). The mean modified skin score was higher in males (18.6 vs 13.9). Sclerodactyly was more frequent in males (90% vs 76%). Active ulceration was present in 33% of males compared to 14% of females (p=0.026). FVC<80% occurred more often in males with 47% compared with 24% in females (p=0.018). Pulmonary hypertension was more common in females with 7% compared to 4% in males. Urine sediment changes were more common in males (8% vs 4%). Gastrointestinal involvement was more common in females (37% vs 29%). Contractures occurred more often in males with 62% compared with 46% in females. Tendon friction rub was observed in 21% of males and 3% of females (p=0.001). Physician global scores of disease activity and damage were higher in males with 48 for both assessments compared to 36 and 30 in females. Conclusion Male patients with jSSc have a higher severity of disease, as it has been reported in adults. 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 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, Flávio R. Sztajnbok: None declared, Ana Paula Sakamoto: 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, Amara Nassar: 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: Infections are a major concern for patients affected by juvenile idiopathic arthritis (JIA) treated with immunosuppressive therapy. Evidence is inconsistent as to whether the start of synthetic or biological disease modifying anti-rheumatic drugs (DMARDs) is associated with an increased risk of serious and at least moderate infection. Objectives: To determine whether the addition of a TNF inhibitor (TNFi) to methotrexate (MTX) may increase the risk of serious and at least moderate infections in JIA patients included in the Pharmachild registry.(1) Methods: Serious and at least moderate infections were analysed in JIA patients, enrolled in the Pharmachild registry at September 30th, 2018, who started as first drug with MTX. We divided patients in 3 treatment groups: “MTX alone”, in which patients had received MTX as the only drug all over their history; “MTX Start”, in which patients had received MTX as first drug; “MTX+TNFi” for those patients who received a TNFi in addition to MTX after a period of “MTX Start”. All the 3 groups were pure, since they received only these drugs. We considered initial infections as related to the treatment if the infection occurred in the drug period or within 90 days after treatment stop.(2) For the group “MTX Start”, infection was related to treatment if occurring in the drug period stopped as soon as the second drug was introduced. For the group “MTX+TNFi”, we considered all the possible correlations between start and end dates of the two drugs, including the time lag of 90 days after any treatment stop. If the interval between two drugs was shorter than 90 days, treatment was considered continuous. Crude rates (number of infections divided by drug exposure, excluding off-drug periods) and true incidence rates (number of first infections divided by the time lag between first drug administration and the date of the infection if the patient experienced the infection, the last Pharmachild visit if the patient didn’t experience the event) were calculated. Results: We enrolled in Pharmachild a total of 8061 patients who experienced 1686 infections. We excluded 41 patients who had infections before any treatment start. Of the final number of 8020 patients, we considered: 1226 patients in the group “MTX alone”, 3128 in the group “MTX start” and 1026 in the group “MTX+TNFi”. 7.7% of the patients in the “MTX alone” group, 2.7% of the patients in the “MTX Start” group and 7.0% of the patients in the “MTX+TNFi” group experienced at least one infection. Crude rates of infections per 1000 person-years resulted: 48.0 for the group “MTX alone”, 22.0 for “MTX Start”, 74.0 for “MTX+TNFi”. Incidence rates per 1000 person-years were: 32.0 for the group “MTX alone”, 17.0 for “MTX Start”, 59.5 for “MTX+TNFi”. The percentage of drug exposure on the patient follow-up was variable among the 3 treatment groups (from 15.6% for the “MTX+TNFi” group to 51.5% for the “MTX alone” group). Conclusion: Pharmachild showed, through the analysis of pure treatment groups, that the addition of the anti-TNF biologic to MTX even triples the incidence rate of infections. References: [1] Swart, et al. Arthritis Res Ther. 2018;20:285; (2) Dixon WG, et al. Arthritis&Rheumatism 56:2896–2904. Disclosure of Interests: Gabriella Giancane: None declared, Joost F. Swart: None declared, Nikolay Tzaribachev: None declared, Nadina Rubio: None declared, Ruben Cuttica Grant/research support from: Roche, Pfizer, Lilly, Bristol Myers Squibb, Novartis, Sanofi Aventis, UCB, Janssen., Consultant for: Roche, Pfizer, Lilly, Bristol Myers Squibb, Novartis, Sanofi Aventis, UCB, Janssen., Speakers bureau: Roche, Pfizer, Lilly, Bristol Myers Squibb, Novartis, Sanofi Aventis, UCB, Janssen., Ingrida Rumba-Rozenfelde: None declared, Wafaa Mohammed Saad Suwairi: None declared, Calin Lazar: None declared, Yosef Uziel: None declared, Albena Telcharova: None declared, Tadej Avcin: None declared, Angela Minaici: None declared, Claudio Len: None declared, Stella Maris Garay: None declared, Alina Boteanu: None declared, Angela Pistorio: None declared, Nico Wulffraat: None declared, 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 (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Speakers bureau: Received honoraria for consultancies or speaker bureaus (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda.

Background: Despite being considered as a distinct diagnostic category in the current ILAR classification criteria, Juvenile Psoriasic Arthritis (JPsA) is known to be a heterogeneous clinical entity, with growing evidence suggesting at least two age-based distinct subgroups(1) Objectives: To identify and characterize subgroups of patients classified as JPsA according to the ILAR criteria and their possible differences in outcomes. Methods: Cross-sectional data from patients enrolled in The EPidemiology, treatment and Outcome of Childhood Arthritis (EPOCA) study and classified as JPsA according to ILAR criteria (n=308) were analyzed. Latent class analysis (LCA) was used to identify subgroups of subjects with similar profiles ILAR criteria for JPsA (presence at onset of psoriasis, dactylitis, nail changes, first-degree relative with psoriasis) and age of arthritis onset. Multinomial logistic regression (three-step method) was performed to explore differences across the obtained classes in clinical-laboratoristic features at onset and outcomes measures collected at visit, namely JADAS scores, VAS-measured Pain, Overall Well-Being (PGA) Pediatric Rheumatology Quality of Life Scale (PRQL). In patients with disease duration more than 2 years (n=233), the relation with Articular and Extraarticular Juvenile Arthritis Damage Index (JADI) was also assessed. Results: LCA revealed 5 classes: 1) late-onset patients with psoriasis, characterized by higher frequency of axial involvement at visit (n = 121); 2) early-onset patients with psoriasis, more likely to be ANA-positive (n = 66); 3) young females with dactylitis at onset and family history of psoriasis, more likely to present with symmetric joint involvement (n = 62); 4) subjects with nail changes and family history of psoriasis(n=34); 5) patients exhibiting dactylitis and nail changes at onset, mostly males, with higher rates of HLA-B27 positivity, small joint involvement and enthesitis at visit (n = 25). Class 1 is associated with higher scores of JADAS10, pain, PGA and JQ; these group also shows higher JADI-A than Class 3. Extraarticular damage is worst for Class 2 subjects. Conclusion: The data driven clustering approach revealed several subgroups, confirming the heterogeneity of JPSA in a multinational cohort. Later-onset subjects with psoriasis have more aggressive disease, being clearly distinct from early-onset ANA-positive patients with psoriasis. The results suggest the need to revise the current classification in order to identify groups that may benefit from different therapeutic choices. References [1] Stoll ML, Nigrovic PA. Clin Dev Immunol. 2006;13(2–4):377–80. Disclosure of Interests: Hala Etayari: None declared, Arūnė Ramanauskiene: None declared, Alessandra Alongi: None declared, Yaryna Boyko: None declared, Yosef Uziel: None declared, Tadej Avcin: None declared, Pierre Quartier Consultant for: AbbVie, Chugai-Roche, lilly, Novartis, Novimmune, Sanofi, and SOBI, Consultant for: AbbVie, Chugai-Roche, Lilly, Novartis, Novimmune, Sanofi, and SOBI, Speakers bureau: AbbVie, BMS, Chugai-Roche, Novartis, Pfizer, and SOBI, Speakers bureau: AbbVie, BMS, Chugai-Roche, Novartis, Pfizer, and SOBI, 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 (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Speakers bureau: Received honoraria for consultancies or speaker bureaus (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Angelo Ravelli Grant/research support from: Angelini, AbbVie, Bristol-Myers Squibb, Johnson & Johnson, Novartis, Pfizer, Reckitt Benkiser, and Roche, Consultant for: Angelini, AbbVie, Bristol-Myers Squibb, Johnson & Johnson, Novartis, Pfizer, Reckitt Benkiser, and Roche, Speakers bureau: Angelini, AbbVie, Bristol-Myers Squibb, Johnson & Johnson, Novartis, Pfizer, Reckitt Benkiser, and Roche, Alessandro Consolaro Grant/research support from: AbbVie, Pfizer,

To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood‐onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRIcSLE).

In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians’ experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.

Abstract Recurrent myocarditis is rare with only few reports having been published for paediatric cases. Repeated use of extracorporeal membrane oxygenation is also uncommon. In this paper we will present a very rare case of a 7-year old girl with recurrent fulminant myocarditis with heart failure requiring cardiopulmonary resuscitation and mechanical circulatory support with extracorporeal membrane oxygenation. Both episodes were precipitated by a viral upper respiratory tract infection, and in both cases the cardiac function eventually completely recovered. The second episode of fulminant myocarditis was particularly complex with markedly elevated markers of myocardiocytolysis, multiorgan dysfunction and the need for prolonged mechanical circulatory support. Nevertheless, the patient made a remarkable recovery. A comprehensive diagnostic workup pointed towards an aberrant immune response as the likely cause of the girl’s susceptibility for fulminant myocarditis.

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ’ and clinicians’ unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union’s Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ’ preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.

The term ‘undifferentiated connective tissue disease’ (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.

OBJECTIVES The European Single Hub and Access point for paediatric Rheumatology in Europe initiative aimed to optimize care for children with rheumatic diseases. Kawasaki disease (KD) is the most common cause of acquired heart disease in children and an important cause of long-term cardiac disease into adulthood. Prompt diagnosis and treatment of KD is difficult due to the heterogeneity of the disease but is crucial for improving outcome. To date, there are no European internationally agreed, evidence-based guidelines concerning the diagnosis and treatment of KD in children. Accordingly, treatment regimens differ widely. The aim of this study is to provide consensus-based, European-wide evidence-informed recommendations for diagnosis and treatment of children with KD. METHODS Recommendations were developed using the EULAR's standard operating procedures. An extensive systematic literature search was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of international experts via online surveys and subsequently discussed in three consensus meetings, using nominal group technique. Recommendations were accepted when ⩾80% agreed. RESULTS In total, 17 recommendations for diagnosis and 14 for treatment of KD in children were accepted. Diagnostic recommendations included laboratory and imaging workup for complete as well as incomplete KD. Treatment recommendations included the importance of early treatment in both complete and incomplete KD, use of intravenous immunoglobulin, aspirin, corticosteroids for high-risk cases, and other treatment options for those with resistant disease. CONCLUSION The Single Hub and Access point for paediatric Rheumatology in Europe initiative provides international evidence-based recommendations for diagnosing and treating KD in children, facilitating improvement and uniformity of care.

OBJECTIVES The European initiative Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) aimed to optimize care for children with rheumatic diseases. Systemic vasculitides are very rare in children. Consequently, despite recent advances, paediatric-specific information is sparse. The lack of evidence-based recommendations is an important, unmet need. This study aimed to provide recommendations for diagnosing and treating children with rare forms of childhood systemic vasculitis. METHODS Recommendations were developed by a consensus process in accordance with the European League Against Rheumatism standard operating procedures. A systematic literature review informed the recommendations, which were devised and evaluated by a panel of experts via an online survey, and two consensus meetings using nominal group technique. Recommendations were accepted when ⩾ 80% of experts agreed. RESULTS Ninety-three relevant articles were found, and 78 recommendations were accepted in the two consensus meetings. General, cross-cutting recommendations and disease-specific statements regarding the diagnosis and treatment of childhood-onset PAN, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and Takayasu arteritis are provided. CONCLUSION These Single Hub and Access point for paediatric Rheumatology in Europe recommendations were formulated through an evidence-based consensus process to support uniform, high-quality standard of care for children with rare forms of paediatric systemic vasculitis.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians’ and patients’ unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient’s unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

The Paediatric Rheumatology European Society (PReS) has over many years, developed a portfolio of educational activities to address increasing educational needs of workforce and support young clinicians to acquire skills to develop new knowledge and deliver clinical care in the future. These educational activities aim to facilitate growth of paediatric rheumatology and ultimately improve the clinical care for children and families. This article describes the current portfolio of PReS educational activities and their relevance to the international paediatric rheumatology community.

To describe the frequency and types of disease damage occurring with childhood‐onset systemic lupus erythematosus (SLE) as measured by the 41‐item Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), and to assess the SDI's ability to reflect damage severity.