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Background Robust scientific evidence on the safety and efficacy of certain vaccines in patients with rheumatic diseases is available; therefore guidelines regarding immunizations in this vulnerable group of children are published via a variety of official health organizations/societies including the Greek Rheumatology Society. Nonetheless, the uptake of specific immunizations is suboptimal. In Greece, vaccinations are mainly delivered by primary care Paediatricians. Objectives The purpose of this study was to describe the knowledge, attitude and current practice of General Paediatricians working in primary care regarding vaccination in children with rheumatic diseases on immunosuppressive medication across Greece and to identify barriers and facilitators that could be used to promote uptake. Methods This was a cross-sectional survey, conducted with an anonymous questionnaire of 25 items distributed to Paediatricians via an online platform. Data collected included demographics, questions on knowledge, perceptions and opinions as well as advice given to families. Additionally, questions addressed three specific categories: live-vaccines, non-live vaccines and annual influenza vaccine. This study was approved by the “P. & A. Kyriakou” Children’s Hospital Research and Ethics Committee. Results Out of 400 questionnaires sent out, 256 were returned. Mean age was 48 years age ((+/-8.2) mean duration of working as a Pediatrician was 15 years (+/-6.7). 25% of the responders worked in rural areas and the remaining in urban areas. 67% worked in the private sector. The majority (78%) of doctors felt that vaccination in children with rheumatic diseases is of pivotal importance. 50% gave specific advice on immunisations at initial diagnosis and 25% checked vaccination status at regular intervals. Responders were using a variety of guidelines in order to reach a clinical decision; still 45% were unaware of the existing national guidelines. 50% were hesitant to adhere to the national vaccination scheme without expert input. Reasons were: not convinced from current literature that the vaccine is safe (32%), afraid to cause disease flare (43%), unable to deal with parental concerns/refusal (54%). 12% of responders felt that the rheumatic disease may have been triggered by a vaccine. The majority (95%) were pro annual influenza vaccination, while a minority (15%) was against live vaccines administration even if the patient was not on immunosuppressive treatment. 75% of doctors were keener to administer booster doses rather than primary ones, whlie 75% of respondents would postpone vaccinations in all cases if disease was active. Conclusion Variation in opinion and clinical practice exists. Overall, although Greek Paediatricians are well informed regarding efficacy and side effects of immunizations, there are steps to be made form principle to practice. Further research will allow the development of clear guidelines to aid in the management of increasing numbers of children with rheumatic diseases. Acknowledgement None Disclosure of Interests None declared

Background Macrophage activation syndrome (MAS) is a severe complication of rheumatologic conditions, mainly systemic juvenile idiopathic arthritis (sJIA), and is classified as a secondary form of hemophagocytic lymphohistiocytosis (HLH). Thrombotic microangiopathy (TMA) is a heterogeneous group of potentially fatal diseases characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ injury. The association between TMA and HLH has been described only in single reports in renal transplant recipients1 and in two cases of virus-induced HLH2. TMA associated with MAS has never been reported so far Objectives To present the preliminary data from a multinational cohort of pediatric patients with MAS and TMA Methods The clinical charts of patients with MAS were retrospectively reviewed to identify the instances that were associated with TMA. Demographic, clinical and laboratory features at MAS and TMA onset, therapeutic interventions and outcome were collected. Results A total of 14 patients, 71.4% females, with MAS and TMA were collected. An underlying rheumatologic disease was reported in 9/14 (8 sJIA or sJIA-like illness and 1 systemic lupus erythematosus). The median age at MAS onset was 8.6 years. In 5 patients MAS and TMA occurred simultaneously, whereas in 4 TMA preceded and in 5 followed MAS. The main features at MAS and TMA onset are presented in Table. Low complement levels and reduced ADAMTS13 activity were observed in 75% and 43% of patients respectively. For MAS management, 85.7% of patients received high-dose corticosteroids and 71.4% cyclosporine; in 5 cases anakinra was added. TMA episodes were treated with plasma-exchange in 57.1% of patients; 7 patients were given biologics (1 rituximab and 6 eculizumab) with good results. Admission to the Intensive Care Unit was required in 85.7% of cases. All patients survived. Conclusion The association of MAS and TMA is a life-threatening condition, likely under-recognized. Clues to suspect TMA in a patient with MAS are the increase in LDH and decrease in platelet count out of proportion of other laboratory abnormalities, the drop in haptoglobin level, the finding of schistocytes in blood smear and the new onset of hematuria. Biologics, particularly rituximab and eculizumab, may offer an adjunctive therapeutic option for refractory cases. References [1] Esmaili H, et al. An update on renal involvement in hemophagocytic syndrome. J Nephropathol 2016; 2Fraga-Rodriguez GM, et al. Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection. BMJ Case Rep2017. Disclosure of Interests Francesca Minoia: None declared, Jessica Tibaldi: None declared, Valentina Muratore: None declared, Romina Gallizzi: None declared, Claudia Bracaglia: None declared, Alessia Arduini: None declared, Elif Comak: None declared, Olga Vougiouka: None declared, Ralf Trauzeddel : None declared, Giovanni Filocamo: None declared, Concetta Micalizzi: None declared, 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