Objective Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League Against Rheumatism)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric SLE patients. Methods Pediatric SLE patients (n=262; 80.9% female) were included from three different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion. Results The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. 18 SLE patients met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (13/18; 72.2%). Eight SLE patients fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (6/8; 75%). Conclusion This is the largest cohort study of pediatric SLE testing the performances of all three classification criteria. The SLICC 2012 criteria yielded the best sensitivity, while the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.

Objective The aim of this study was to conduct a detailed ophthalmological examination in children with systemic lupus erythematosus (jSLE), including choroidal thickness (ChT), choroidal vascularity index (CVI) and peripapillary retinal nerve fiber layer (RNFL). Methods The study included all jSLE patients ( n = 21) diagnosed according to the Systemic Lupus International Collaborating Clinics classification criteria between January 2017 and April 2017, and an age- and gender-matched control group ( n = 21). The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity. After routine eye examinations, ChT at five points (750 µ and 1500 µ from the center of the fovea both in the temporal and nasal quadrants and under the fovea), total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), CVI and RNFL thickness at the optic disc were evaluated. Results One patient had active ocular involvement in the form of episcleritis. Another patient had corticosteroid-induced cataract. The median age of the patients was 16 years (6-19 years). ChT at five points, TCA, LA and SA were found to be higher in patients with jSLE, whereas RNFL thickness and CVI were similar to those of the healthy control individuals. No correlation was determined between optical coherence tomography findings, SLEDAI and the immunological parameters (antinuclear antibodies, anti-double-stranded DNA, complements 3 and 4, extracted nuclear antigen antibody, antiphospholipid antibody). Intraretinal and subretinal fluid was not present in any of the patients. Conclusion The choroid was thicker in patients with jSLE than in the control group. The study results suggest that jSLE may affect the choroid. Ophthalmological evaluation is important in SLE patients, even in the absence of relevant complaints.

Background Coronavirus disease 19 (COVID-19) may have a severe course in children. Multisystem inflammatory syndrome in children (MIS-C) is the post-COVID complication characterized by an exaggerated inflammation, observed in children. However, data on the underlying pathophysiology are sparse. We therefore aimed to assess the cytokine and chemokine profiles of children with MIS-C and compare these to life-threatening severe SARS-CoV-2 and healthy controls (HCs) to shed light on disease pathophysiology. Methods Samples of 31 children with MIS-C, 10 with severe/critical COVID-19 and 11 HCs were included. Cytokine and chemokine profiles were studied and compared in between groups. Results Most cytokines and chemokines related to IL-1 family and IFN-γ pathway (including IL-18 and MIG/CXCL9) and IL-17A were significantly higher in the MIS-C group when compared to the severe/critical COVID-19 group and HCs. IP-10/CXCL10 and IL-10 were higher in both MIS-C patients and severe/critical COVID-19 compared to HCs. Conclusion Our results suggest that IL-1 and IFN-γ pathways play an important role in the pathophysiology of MIS-C. Impact This study defines a pattern of distinctive immune responses in children with MIS-C and in patients with severe/critical COVID-19. As the COVID-19 pandemic continues, biomarkers to identify MIS-C risk are needed to guide our management that study results may shed light on it.

Familial Mediterranean fever (FMF) is the most common autoinflammatory disease in the world. The most serious complication of FMF is the development of secondary amyloidosis. Besides genetic factors, environment has been implicated in the development of this complication. The main objective of this study is to analyze whether there has been a substantial decrease of secondary amyloidosis in Turkey and possible effective factors. For this purpose, clinical features of the patients diagnosed with secondary amyloidosis between the years 1978 and 1990 were compared with those diagnosed between 2000 and 2009. Severity scores were determined by the use of a scoring system modified for children. Median ages of the group diagnosed between 1978 and 1990 (n = 115; 12.1% among a total of 947 renal biopsies) and diagnosed after 2000 (n = 19; 2% among a total of 974 renal biopsies) were 12 and 13 years, respectively. There were no significant differences between the two patient groups according to gender, age, age of onset, disease duration, and disease severity. There was, however, a clear decrease in the percentage of biopsies with secondary amyloidosis from 12.1% (1978-1990) to 2% (after 2000; p < 0.001). Our results have shown that there has been a significant decrease in the rate of secondary amyloidosis in Turkey. The main reason for this decrease is better medical care with increased awareness and treatment of the disease. However, we suggest that the improvement of infectious milieu may possibly have had a positive effect on the course of this monogenic disease, since inflammatory pathways related to innate immunity are deregulated.

Objective. Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK.Methods. We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients. Results. Twenty-five paediatric-onset (<18 years of age at diagnosis) and 154adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during followup, whereas cerebrovascular disease was more common in adult patients. Conclusion. Our paediatric onset TAKpatients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes. Methods Study protocol and patientsWe retrospectively evaluated the medical records of TAK patients followed at

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disease characterized by sterile bone inflammation. It is an orphan disease with many unclear aspects in terms of diagnosis, treatment and follow-up. The aim of this study was to report our experience of pediatric CRMO patients. Children who were diagnosed with CRMO, and were followed-up between January 2008 and January 2017, were included in this study. There were 15 CRMO patients (8M/7F) with a median age at diagnosis of 9.0 years (range: 0.6-15.0). Bone pain was the most common presenting symptom. All of the patients had multifocal bone lesions. Vertebrae (66.7%) and femur (66.7%) were the most commonly affected bones. Eight of the patients also had sacroiliitis; however, only one of them was HLA-B27 positive. Whole-body magnetic resonance imaging (MRI) was used as a diagnostic tool in 13 patients revealing bone marrow edema (84.6%), osteitis (69.2%), and periosteal reaction (61.5%). All patients were initially treated with non-steroidal anti-inflammatory drugs (NSAIDs), however, disease-modifying anti-rheumatic drugs, anti-TNF agents or pamidronate were added to therapy due to inadequate treatment response. Clinical remission was achieved in 12 patients (1 with NSAIDs, 3 with methotrexate, 1 with pamidronate and 7 with an anti-TNF agent). During the follow-up period, relapses were observed in four patients who presented with pain and/or a newly formed bone lesion on MRI. Eventually, however, all of these patients also reached remission. CRMO is a chronic disease which may have a progressive or relapsing-remitting course. Improvement of the knowledge about this rare disease may help to enlighten the unknowns of the disease.

Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease in the world. The disease characteristics may vary in different age groups. In this study, we aimed to compare disease characteristics and treatment compliance according to the age of pediatric FMF patients. This is a single-center, cross-sectional study. Between August and October 2016, patients who were diagnosed with FMF, participated to the study. 378 pediatric FMF patients were enrolled in the study. Among those, age at symptom onset was ≤ 5 years in 69%, 6-11 years in 26% and ≥ 12 years in 5%. Patients older than 12 years old at symptom onset, had significantly less frequent fever attacks than the patients from other age groups. Patients younger than 5 years old at symptom onset had significantly higher international severity scoring system for FMF (ISSF) than other patients. And M694V homozygosity was significantly more frequent in patients with younger age at symptom onset. Patients younger than 5 years old were using their drugs more regularly than the other age groups (p = 0.002). Drug compliance was 90.5% in patients ≤ 5 years, 64.4% in patients 6-11 years, 58.3% in patients ≥ 12 years. The disease characteristics of FMF may differ between patients with different age at symptom onset. Younger age at disease onset seems to be related with more severe course; thus these patients should be followed-up more closely. In addition, treatment compliance which is critical for prevention of amyloidosis in FMF should be questioned especially in adolescent patients.

BackgroundBehçet’s disease (BD) is an autoinflammatory disorder which may present with multi-systemic involvements including the vascular, cutaneous, articular, gastrointestinal, and/or central nervous systems (CNS). CNS involvement appears in two main groups: parenchymal or non-parenchymal. Neurological symptoms in children and adolescents can be confused with many other disorders and may be the initial symptom of BD.ObjectivesTo report our experiences of the juvenile Behçet’s Disease patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of Juvenile Behçet’s Disease with CVST.MethodsThe patients who met Pediatric Behçet’s Disease (PEDBD) classification criteria for juvenile Behçet’s Disease from 3 referral centers in Turkey were reviewed retrospectively. Disease activity was assessed by BD current activity form (BDCAF). A systematic review of literature of all published data was conducted.ResultsThe study group consisted of 12 juvenile BD patients with CVST. At the time of CVST diagnosis, the most common symptom was headache (%100), followed by vomiting (25%), blurred vision (16.6-7%), and disturbances in eye movements (16.7%). Six (50%) patients presented with sinus venous thrombosis as an initial symptom. Transverse sinus was the most frequently affected sinus (9/12, 75%) followed by superior sagittal sinus (8/12, 66.6%) and sigmoid sinus (1/12, 8.3%). The median (minimum-maximum) BDCAF was 6 (5-8). Four children (33.3%) had another venous thrombosis apart from CVST. All patients received pulse methylprednisolone for three consecutive days continued with oral prednisolone. Steroid treatment was tapered and discontinued minimum in six months. Eleven patients received azathioprine concomitant to steroid treatment at the time of CVST. All the patients received anticoagulant therapy concomitantly. Only one patient had relapse. Median (min-max) follow-up period was 4 years (1-10). In the literature review, we identified nine articles, describing 35 pediatric CVST patients associated with BD. Thirty patients achieved remission, while five patients had residual neurologic deficit.ConclusionFurther multicenter studies with more patients and prospective follow-up may help us to understand the whole spectrum in these patients.References[1] Behcet, H., über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatologische Wochenschrift, 1937(105): p.1152-63.[2] Metreau-Vastel, J., et al., Neurological involvement in paediatric Behcet’s disease. Neuropediatrics, 2010. 41(5): p.228-34.Disclosure of InterestsSelcan Demir: None declared, Ceyhun Acarı: None declared, özge Basaran: None declared, Erdal Sag: None declared, Kader Karlı Oğuz: None declared, Yelda Bilginer: None declared, Erbil Unsal Grant/research support from: Novartis, AbbVie, Roche, Koçak Pharma, Speakers bureau: Novartis, AbbVie, Roche, Koçak Pharma, Seza özen Consultant for: Seza Ozen is receiving consultancy fees from Novart...