Explore the vast range of titles available.

Vaccination programs are effective strategies in preventing infectious diseases and controlling epidemics. Vaccination against SARS-CoV-2 in children has not yet been approved globally, and it is unclear what attitude families will take when it is approved in children. We aimed to investigate the underlying causes of vaccine acceptance, hesitation, and refusal, as well as concerns about the acceptability of the COVID-19 vaccine by parents of children with rheumatic diseases. Parents of children followed up with a diagnosis of rheumatic disease in the pediatric rheumatology outpatient clinic of a university hospital were included in the study. We applied a closed web-based online survey conducted cross-sectionally and sent to the participants via mobile smartphones. For fathers, mothers, and their children, acceptance rates for a COVID-19 vaccine were 64.2%, 57.7%, and 41.8%, respectively. In the multivariate analysis, factors affecting parents' acceptance of vaccines for their children were as follows: “Receiving antirheumatic medications regularly (AOR 5.40, 95% CI 1.10–26.33, p = 0.03), the previous history of getting special recommended vaccines (AOR 4.12, 95% CI 1.12–27.85, p = 0.03), relying on vaccines for ending pandemic (AOR 8.84, 95% CI 2.80–27.85, p = 0.001), complying with the pandemic measures entirely (AOR 5.24, 95% CI 1.46–18.74, p = 0.01)“. The two most common reasons for vaccine rejection were fear of the side effects of the vaccine and its possible interaction with rheumatic drugs used by children. According to our survey, parents were more likely to accept a COVID-19 vaccine for themselves than their children. The success of COVID-19 vaccination programs sources highly on people's willingness to accept the vaccine. It is crucial to vaccinate children for achieving herd immunity and in terms of avoiding vaccine hesitancy. Larger data examining the causes of concerns in parents of both healthy children and children with chronic diseases should be delineated.

ObjectivesMulti-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission.MethodsThe MIS-C patients under 18 years old diagnosed and treated in three referral centers between July 2020 and March 2021 were included. Data of the patients were retrospectively obtained from their medical records.ResultsOverall, 76 subjects (24 females) with a mean age of 8.17 ± 4.42 years were enrolled. Twenty-seven (35.5%) patients were admitted to the PICUs. The two most common systemic involvement patterns were cardiac and gastrointestinal. There was only one lethal outcome in a patient with underlying acute lymphoblastic leukemia. Those with higher procalcitonin levels at admission were found to stay longer in the hospital (r = 0.254, p = 0.027). The risk of PICU admission increased with age (aOR: 1.277; 95% CI: 1.089–1.498; p = 0.003) and with decreased initial serum albumin levels (aOR: 0.105; 95% CI: 0.029–0.378; p = 0.001).ConclusionAlthough there is a wide clinical variability among the patients with MIS-C, we suggest that those with older age and lower initial serum albumin levels merit close monitoring due to their higher risk for PICU admission. Key Points• Although there is a wide variability regarding the management process among clinicians, MIS-C is a rare, severe, less understood complication of COVID-19 that may cause rapid clinical deterioration in the patients.• Clinicians should be aware of this condition in children with persistent fever and a family history of COVID-19.• Older age and low serum albumin levels are the independent predictors for the pediatric intensive care unit admission among MIS-C patients.

•While body weight and body mass index were similar in the patient and control groups, height was different to the detriment of the patient group.•The rate of high disease activity at the last visit in overweight children was found to be three times higher than in children with normal BMI•It has been established that high disease activity can negatively affect body weight and BMI.•The patient and control groups were also evaluated for energy, macro- and micronutrient intake characteristics across JIA subtypes. Proper nutrition is a significant contributor to growth achievement in patients with juvenile idiopathic arthritis (JIA). In this study, the aim was to analyze the growth parameters and nutritional status of children with JIA and then compare them with their healthy peers. A cross-sectional study was conducted with 54 patients with JIA and the same number of healthy peers. Growth parameter z-scores and nutrient distributions were analyzed and compared with a control group and among disease subgroups. While the average height in the control group was significantly greater than in the patient group, there was similarity in terms of body weight and body mass index (BMI) (P < 0.001, P = 0.33, P = 0.14, respectively). Body weight and BMI z-scores of patients with high disease activity at the most recent visit were significantly lower (P = 0.03, P = 0.01, respectively). Both groups had similar energy and protein requirement–meeting percentages (P = 0.62, P = 0.51). JIA atients had higher carbohydrate intake (P = 0.04), and fat intake was higher in controls (P = 0.02). Energy obtained from junk food was higher in patients with entesitis-related arthritis (ERA) compared to oligoarticular JIA and polyarticular JIA (P = 0.03). Micronutrient intake in the ERA group was significantly lower for vitamin E, C, and folate (P = 0.02, P = 0.03, P < 0.001). In our cohort, patients had a lower height score. As they have a diet characterized by adequate energy/protein, carbohydrate, and high fat intake, this may be a reflection of disease activity. Although some of the micronutrient intakes were less than normal in both groups, significant deficiencies were identified in the ERA group.

Objective We aimed to find out the asymptomatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) seroprevalence among pediatric patients with rheumatic diseases and healthy children and to compare them with each other. Methods Patients with familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), and juvenile systemic lupus erythematosus (jSLE) and healthy children as healthy control (HC) group who remained asymptomatic during the pandemic are examined by ELISA immunoglobulin (Ig) A and IgG tests in this cross-sectional study. Results Overall, 149 subjects (90 females) were included in the study. While IgA was positive in 15 subjects (10%) (HC: 8, jSLE: 3, FMF: 2, JIA: 2; p  = 0.196), IgG was positive in 14 subjects (9.4%) (HC: 7, JIA: 5, FMF: 1, jSLE: 1; p  = 0.156). Nineteen subjects (12.75%) were IgA or IgG positive (HC: 8, JIA: 5, jSLE: 3, FMF: 3; p  = 0.644). Although not significant, seropositivity was more often in HC group. Both IgA and IgG positivity were not found to be related to age, sex, underlying rheumatic diseases, and received treatments of the patients. Conclusion We revealed that patients with childhood-onset rheumatic diseases, even if they receive immunosuppressive medication such as biologic or conventional disease-modifying anti-rheumatic drugs, might have an asymptomatic SARS-CoV-2 infection, similarly to their healthy peers. Key points • Although it has been already known that children are most likely to have asymptomatic SARS-CoV-2 infection, there is a lack of data on the disease course of children with rheumatic disease. • There was no significant difference regarding the asymptomatic SARS-CoV-2 seropositivity rates between healthy children and the patients with childhood-onset rheumatic diseases. • Patients with childhood-onset rheumatic diseases, even if they receive immunosuppressive medication, might have asymptomatic SARS-CoV-2 infection, similarly to their healthy peers.

Background Enthesitis-related arthritis (ERA) may exhibit a distinct disease spectrum on the basis of ethnic origin. The pediatric rheumatology teams from the Istanbul Medical Faculty and Tunisia Kassab Institute engaged in collaboration via the Second Sister Hospital Initiative of the European Society of Pediatric Rheumatology (PReS) to investigate the clinical characteristics and outcomes of children with ERA. Methods The medical records of patients with the diagnosis of ERA were reviewed retrospectively. The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was the tool for assessing disease activity. In addition to clinical and laboratory findings, treatments and disease outcomes were compared. Results A total of 94 children with ERA were enrolled (45 Tunisian, 49 Turkish). Sex and age at disease onset were similar between the groups. Heel pain (8.8% vs. 61.2% for Tunisia vs. Türkiye, p  = 0.03) and enthesitis (40% vs. 69.3% for Tunisia vs. Türkiye, p  = 0.03, p  = 0.8) were more common in Turkish children. Conversely, the rates of sacroiliac tenderness, suggesting clinical sacroiliitis (91.1% vs. 55.1% for Tunisia vs. Türkiye), and axial disease (97.8% vs. 55.1% for Tunisia vs. Türkiye) were significantly greater in Tunisian children ( p  = 0.002 and p  < 0.001, respectively). Overall, 45.7% of the cohort was HLA-B27 positive, including 32% of Turkish patients and 60% of Tunisian patients ( p  < 0.001). HLA-B27 positivity did not influence age at disease onset ( p  = 0.45) but was associated with a longer diagnostic delay of the disease ( p  < 0.001). Nearly half of the Turkish children received biologics during the disease course, whereas only 8.9% of the Tunisian children did. While the median JSpADA scores at disease onset were similar between the groups, Turkish patients had significantly lower scores at the last visit than Tunisian patients did ( p  < 0.001). Conclusions This study highlights notable differences in the clinical features and outcomes of ERA among Turkish and Tunisian children, emphasizing the potential influence of ethnic and regional factors on disease presentation and management. Variations in HLA-B27 positivity and treatment approaches, including the use of biologics, further underscore the need for tailored strategies in managing ERA across diverse populations.

BackgroundAlthough colchicine is the mainstay of familial Mediterranean fever (FMF) treatment, 5–10% of patients are considered to have colchicine resistance (CR). However, there is no globally agreed CR definition or indications for biological disease-modifying anti-rheumatic drugs (bDMARDs).MethodsA survey on ‘Biologics in Monogenic Autoinflammatory Diseases’, part of the ‘Clinical Practice Strategies’ (CLiPS) initiative, was conducted by a JIR cohort-initiated eCOST network among expert participants worldwide. Our primary aim was to provide a flowchart reflecting the different CR definitions and present data regarding bDMARD indications. The secondary aim was to determine how specific biases influence clinical approaches. We analysed the CliPS according to the experience levels of physicians, country-specific FMF prevalence, countries’ gross domestic product, bDMARD availability and reimbursement policies of the countries.ResultsA total of 223 responses from 46 countries were included in the study. Almost half of the respondents (73/160, 45.6%) indicated that three to four attacks within the preceding 6 months were necessary for their CR definition. The most frequently used acute-phase reactant was C-reactive protein (157/164, 95.7%). Almost three-fourths of the respondents (74%, n=165) considered that supplementary factors, including complications of FMF, attack severity, elevated activity scores, patient-reported outcome and quality of life scales, influenced their CR definition.ConclusionWe present a novel flowchart describing physicians’ general attitudes and unique findings regarding management strategies for colchicine-resistant FMF and shifting trends influenced by epidemiological and socioeconomic factors.

Aim:The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) is the only disease activity score specifically validated for children with enthesitis-associated arthritis (ERA). It was developed to address the need for an effective measurement tool to assess disease activity in this population. We aimed to evaluate the clinical course of patients with ERA using JSpADA and to compare the effects of treatment modalities using JSpADA.Methods:This cross-sectional observational study enrolled 61 patients with ERA who were followed up between January 2020 and 2023. Clinical features, treatment options, and JSpADA were noted in electronic medical files. The effectiveness of treatment modalities was compared by JSpADA.Results:The median age of onset of the group was 10 [interquartile range (IQR), 9-15] years. The study cohort included three groups of patients: 1) DMARD received (n=34); 2) biologic drug received (n=14); 3) DMARD and biological combination received (n=13). Forty-three cases (70%) presented with peripheral arthritis, including enthesitis, whereas 18 (30%) patients had axial involvement. At disease onset, the median JSpADA scores were 2 (IQR, 2-3), 2.5 (IQR, 2-3), and 3.5 (IQR, 2.5-5) in groups 1, 2, and 3, respectively (p=0.27). At the first year of follow-up, there was a significant improvement in the disease activity of groups 1 and 2 (p=0.02 and p=0.04). However, there was no significant reduction in JSpADA values in the third group.Conclusion:In patients with ERA, intermittent JSpADA evaluation during visits can guide the objective and accurate follow-up and treatment response of patients.

Background There is no clear data on the optimal duration of treatment with anti-interleukin-1 drugs in colchicine-resistant familial Mediterranean fever patients, as well as on the dose interval. This study aimed to assess patients whose canakinumab dose interval was adjusted according to a specific protocol, with the objective of evaluating the effectiveness of implementing this protocol for the patient care. Methods The files of 45 patients whose canakinumab treatment interval was opened with a standard protocol previously determined by the Delphi method were retrospectively reviewed. Results Canakinumab treatment was initiated once a month for all patients. In the sixth month of canakinumab treatment, a dose interval extension was introduced; however, 7 patients (15.5%) experienced an attack, and consequently, no further interval extension was administered to them. For 29 patients, the dose interval was successfully extended to once every three months, as they remained attack-free for a year after the first interval extension. Nine patients continued receiving the drug every 2 months, as they had not yet completed one year since the first extension. The study found no significant correlation between experiencing an attack during the dose interval extension protocol and the number, duration of attacks, or autoinflammatory diseases activity index score. Conclusion Extending treatment intervals with canakinumab in colchicine-resistant familial Mediterranean fever shows promise for favorable outcomes.

Background: The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course. Methods: This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group. Results: Four subgroups were identified as follows: cluster I, predominantly Kawasaki-like features (n = 100); cluster II, predominantly MAS-like features (n = 34); cluster III, predominantly LV dysfunction (n = 47); cluster IV, other presentations (n = 112). The duration of fever was longer in cluster II and the length of hospitalization was longer in both clusters II and III. Laboratory findings revealed lower lymphocyte and platelet counts and higher acute phase reactants (APRs) in cluster II, while patients in cluster IV showed less inflammation with lower APRs. The resolution of abnormal laboratory findings was longer in clusters II and III, while it was shortest in cluster IV. Seven patients died. Among them, four belonged to cluster II, while three were labeled as cluster III. Patients with severe course had higher levels of neutrophil–lymphocyte ratio, mean platelet volume, procalcitonin, ferritin, interleukin-6, fibrinogen, D-Dimer, BNP, and troponin-I, and lower levels of lymphocyte and platelet counts. Conclusion: As shown, MIS-C is not a single disease presenting with various clinical features and outcomes. Understanding the disease spectrum will provide individualized management.

Classical attacks of familial Mediterranean fever (FMF) are often accompanied by fever, but some of the patients have attacks without fever. This study aimed to compare the characteristics of FMF patients with and without fever during their attacks and draw attention to the different clinical presentations of FMF in children. Medical files of patients aged 0–18 years who were followed up with the diagnosis of FMF in two reference pediatric rheumatology centers were reviewed retrospectively. The patients were divided into two groups: children who had had no fever in any of their attacks were assigned as group 1, and those who had fever during their attacks were classified as group 2. Out of 2003 patients evaluated, 191 (9.53%) patients had attacks not accompanied by fever and their median age at onset of symptoms (7.0 vs. 4.0 years, p  < 0.001) and the median age at diagnosis (8.6 vs. 6.0 years, p  < 0.001) were significantly higher; however, group 2 had a delay in diagnosis. The annual number of attacks and abdominal attacks were more common in group 2; arthritis, arthralgia, erysipelas-like rash, exercise-induced leg pain, and myalgia were more common in group 1.     Conclusion: The data from the assessment of children with FMF attacks not accompanied with fever were presented for the first time. Children with late age onset of FMF and dominance of musculoskeletal features may display attacks not accompanied with fever. What is Known: • Familial Mediterranean fever (FMF) is the most common inherited auto-inflammatory disease, characterized by recurrent attacks of fever, serositis, and musculoskeletal symptoms. • Although fever is the most common symptom, few studies have reported attacks without fever. What is New: • The aim of this study was to identify patients with FMF but without fever during attacks and to demonstrate their distinctive presentations. • We found that 7% of our patients had afebrile attacks with predominant musculoskeletal symptoms and were diagnosed earlier than patients with febrile attacks, probably due to early referral to pediatric rheumatology clinics.