BCG infections occur more frequently in patients with underlying primary immunodeficiency disease (PIDD). In this study, we aimed to evaluate the ratio of PIDD in the patients with BCG infections. Patients with BCG infections were analyzed in a tertiary referral centre in the 2015-2020 period. Forty-seven patients with BCGitis/

Macrophage activation syndrome (MAS) is a devastating complication of systemic JIA (sJIA), seen in approximately 10-25% of the sJIA patients. A number of criteria have been proposed to differentiate between activation of sJIA and MAS, including HScore and the recently proposed MS-score. This is the first study comparing the performances of MS-score and HScore for the diagnosis of MAS in sJIA patients. Systemic JIA patients followed at Hacettepe University Pediatric Rheumatology Unit were included in the study. Clinical features and laboratory findings at the time when the disease was most active or patients were diagnosed with MAS were recorded retrospectively. HScore and MS-score were calculated and the diagnostic performance for MAS was compared by receiver operating characteristic (ROC) curve analysis. Seventy-one sJIA patients were included (23 MAS, 48 activation). There was no difference in age of onset (median 4.7 vs. 5.0 years) and gender (73.9% vs. 54.2%) between patients who had MAS and sJIA activation. Median MS-score and HScore were higher in the MAS group. ROC curve analysis revealed that the HScore performed slightly better in diagnosing MAS, compared with the MS-score (AUC = 0.965 and 0.901 for HScore and MS-score respectively, P < 0.001). In our cohort, the optimal cut-off for the MS score was ≥ − 1.64 (sensitivity: 91.3%; specificity: 83.8%) and for the HScore it was ≥ 162.5 (sensitivity: 91.3%; specificity: 90.2%). HScore performed slightly better than MS-score for the diagnosis of MAS in our cohort. Keywords Systemic JIA • Macrophage activation syndrome • Hscore • MS-score Abbreviations MAS Macrophage activation syndrome sJIA Systemic juvenile idiopathic arthritis AOSD Adult-onset Still's disease AUC Area under the curve ROC Receiver operating characteristic Rheumatology INTERNATIONAL Erdal Sag and Armagan Keskin contributed equally to this work.

Pediatric primary antiphospholipid syndrome (APS) is a very rare disease with significant distinctions from the APS in adults. Herein, we present our experience in the diagnosis and treatment of six pediatric primary APS patients, who met the updated Sapporo criteria for the APS diagnosis. One of them was also diagnosed as having probable catastrophic APS (CAPS) due to the involvement of three different organ systems simultaneously. Besides vascular involvement, four patients had thrombocytopenia, one had psychiatric disorder, and one had chorea and valvular heart disease. All patients received immunosuppressive treatment along with long-term anticoagulation therapy. Specific neurologic and hematologic manifestations that are not part of the classification criteria can be seen in children with primary APS. Therefore, using the adult criteria for diagnosing pediatric APS may result in missed or delayed diagnoses in children.

BackgroundAutoinflammatory diseases (AID) are characterized by a dysregulation of innate immunity leading to uncontrolled inflammation. The treatment in AID is critical to control the disease activity, to prevent complications, and to improve the health-related quality of life. Biologic drugs have revolutionized the treatment and outcomes in AID.ObjectivesHerein we aim to present the clinical characteristics of children to whom biologic drug therapy was initiated for the management of AID.MethodsA web-based registry called the Helios Registry (Hacettepe univErsity eLectronIc research fOrmS) has been formed to evaluate the data of all children on biologic treatment. We have been enrolling patients since August 2018 retrospectively and prospectively. We have analyzed the data about the general characteristics of the patients, treatment, the biologic drug used, and adverse effects. Only the patients with the following diagnoses were included: systemic juvenile idiopathic arthritis (SJIA), familial Mediterranean fever (FMF), cryopyrin associated periodic syndrome (CAPS), and chronic recurrent multifocal osteomyelitis (CRMO).ResultsOf 60 patients included, 19 had FMF (31.7%), 24 had sJIA (40%), 10 had CAPS, (16.7%), and 7 had CRMO (11.7%). Their median age was 10.7 (2-20) years old and disease duration was 2.8 (0-6) years, at the time of biologic drug initiation. 58.3% were currently on canakinumab, 20% anakinra, 10% tocilizumab, 10% etanercept, and 1.7% adalimumab. 63.3% of our patients had previously used at least one other biologic drug. The rate of glucocorticoid use before biologic treatment was 56.6%. The median duration of glucocorticoid treatment after initiating biologic drugs was 7.4 months. 56 (93%) patients achieved remission on biologic therapy. There were 15 patients (25%) who received tuberculosis prophylaxis due to positive tuberculin skin test (diameter≥10 mm) and there was no Quantiferon test positivity. Thirteen adverse events (AE) had been noted. 2 of them were serious events as anaphylaxis due to tocilizumab infusions. The rest of the adverse events were mild thrombocytopenia (n=2), varicella infection (n=1), and local side effects (n=8). The median number of the infections per year was one and there were no death or malignancy.ConclusionThe most commonly prescribed biologic drugs were IL-1 inhibitors especially for patients with IL-1-mediated AID (FMF, CAPS, and SJIA). The biologic treatment in AID is effective and there were no serious side effects.References[1] Ozen S, Bilginer Y. A clinical guide to autoinflammatory diseases: familial Mediterranean fever and next-of-kin. Nat Rev Rheumatol. 2014 Mar;10(3):135-47. doi:10.1038/nrrheum.2013.174Disclosure of InterestsSelcan Demir: None declared, Ezgi Deniz Batu: None declared, Fuat Akal: None declared, Erdal Sag: None declared, Ummusen Kaya Akca: None declared, Elif Arslanoğlu: None declared, Emil Aliyev: None declared, Kübra Yüksel: None declared, Armağan Keskin: None declared, Yelda Bilginer: None declared, Seza özen Consultant for: Seza Ozen is r...

BackgroundColchicine has been used in the treatment of Familial Mediterranean Fever (FMF) since 1972. Apart from the inhibiting mitosis in all cells, colchicine has an anti-inflammatory effect by inhibiting activation and migration of neutrophils. Colchicine is a safe drug at recommended doses, but it can cause rare side effects including hematological findings such as lymphopenia, thrombocytopenia and neutropenia.ObjectivesIn this study we aimed to define the adverse effect of colchicine on platelet function and its clinical relevance.MethodsA total of 220 FMF patients between June 2016-2017, followed at Hacettepe University Pediatric Rheumatology Department and were on colchicine treatment for at least one year, were included to the study.ResultsAmong the selected 220 FMF patients, 100 of them (54% female) described hematological symptoms when questioned in detail. The mean age of these patients was 11.74 ± 4.86 years. The mean cumulative colchicine exposure was 5.7±3.8 years. The most common referral symptom was frequent epistaxis (79%) followed by easy bruising (69%), and menstrual disorder including prolonged or heavy menstrual bleeding (21.8% among female patients). Among these 100 patients, 36 of them had prolonged bleeding time and impaired platelet aggregation test. Patients who had abnormal platelet function tests (the group with abnormal bleeding time) were receiving higher colchicine doses (median 0.05 vs 0.03 mg/kg/day; p:0.001) compared to the patients who had normal platelet function tests (bleeding time normal group) However there were no significant difference in terms of cumulative colchicine exposure (median 6.5 vs 4.5 years; p:0.07) and total platelet counts (median 288500 vs 279000/mm3; p:0.61). Patients with abnormal platelet function tests also had more epistaxis (47% vs 7%; p<0.001) bruising (51% vs 3%; p<0.001) and dysmenorrhea (among female patients 100% vs 26%; p<0.001). Colchicine was not reduced in these patients and no life-threatening event was observed.ConclusionIn our study, we have shown prolonged bleeding time for the first time in the literature. Colchicine may cause microtubule inhibition in platelets as well as in other cells and impair platelet function. Further prospective studies are needed to clarify the significance of this side effect.Reference[1] Padeh S, Gerstein M, Berkun Y. Colchicine is a safe drug in children with familial Mediterranean fever. J Pediatr 2012;161(6):1142-1146.Disclosure of InterestsÖzlem Şatırer: None declared, Selcan Demir: None declared, Erdal Sag: None declared, Armağan Keskin: None declared, Yelda Bilginer: None declared, Şule Ünal: None declared, Seza Özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche