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Objective: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA., Methods: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated., Results: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively)., Conclusion: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly., (© Crown copyright 2023.)

Objective: Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF) are autoinflammatory disorders typically characterized by recurrent fever attacks. These recurrent fever attacks can lead to depression and anxiety in mothers of these patients. This study aimed to compare the depression and anxiety levels in mothers of PFAPA and FMF patients., Methods: This study is a cross-sectional observational study. 48 mothers of children with FMF and 70 mothers of children with PFAPA participated in the study. Mothers in these two groups were compared in terms of anxiety and depression by using the validated Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI)., Results: Depression and anxiety scores of mothers were found to be similar in FMF and PFAPA groups. Moderate or high level of anxiety was seen in 32% of mothers of patients with PFAPA and 27% of mothers of patients with FMF. 23% of mothers of patients with PFAPA were evaluated as having moderate or severe depression, and 18% of mothers of patients with FMF were evaluated as having moderate depression. There was no statistically significant difference between the duration, frequency of attacks, recurrent hospitalizations, sociodemographic characteristics, and inventory scores., Conclusion: Depression and anxiety scores of mothers with children diagnosed with FMF and PFAPA are similar. These two diseases affect families psychosocially at similar levels. It is important to provide psychosocial support to families.

Objectives: Colchicine forms the mainstay of treatment in FMF. Approximately 5-10% of FMF patients are colchicine resistant and require anti-IL-1 drugs. We aimed to compare the characteristics of colchicine-resistant and colchicine-responsive patients and to develop a score for predicting colchicine resistance at the time of FMF diagnosis., Methods: FMF patients (0-18 years) enrolled in the Turkish Paediatric Autoinflammatory Diseases (TURPAID) registry were included. The predictive score for colchicine resistance was developed by using univariate/multivariate regression and receiver operating characteristics analyses., Results: A total of 3445 FMF patients [256 (7.4%) colchicine-resistant and 3189 colchicine-responsive) were included (female:male ratio 1.02; median age at diagnosis 67.4 months). Colchicine-resistant patients had longer, more frequent attacks and were younger at symptom onset and diagnosis (P < 0.05). Fever, erysipelas-like erythema, arthralgia, arthritis, myalgia, abdominal pain, diarrhoea, chest pain, comorbidities, parental consanguinity and homozygosity/compound heterozygosity for exon 10 MEFV mutations were significantly more prevalent among colchicine-resistant than colchicine-responsive patients (P < 0.05). Multivariate logistic regression analysis in the training cohort (n = 2684) showed that age at symptom onset, attack frequency, arthritis, chest pain and having two exon 10 mutations were the strongest predictors of colchicine resistance. The score including these items had a sensitivity of 81.3% and a specificity of 49.1%. In the validation cohort (n = 671), its sensitivity was 93.5% and specificity was 53.8%., Conclusion: We developed a clinician-friendly and practical predictive score that could help us identify FMF patients with a greater risk of colchicine resistance and tailor disease management individually at the time of diagnosis., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Objectives: Familial Mediterranean fever (FMF) is an auto-inflammatory disease that causes recurrent episodes of fever, abdominal pain, chest pain, and arthritis. Although FMF is well known, protracted febrile myalgia syndrome (PFMS) is a clinical condition that is rare and difficult to diagnose than other symptoms of FMF. PFMS causes fever, myalgia, and acute phase reactant elevation that lasts 2-4 weeks if corticosteroid treatment is not given. In some cases, fever may not be seen. The purpose of this report is to share our experience with PFMS patients in our clinic., Methods: This is an observational, retrospective, single-centre study. We evaluated patients who had been diagnosed with PFMS at our paediatric rheumatology clinic., Results: Protracted febrile myalgia syndrome was observed in 14 patients. Nine of the patients were female. The median age at the time of diagnosis of PFMS was 10 years. Only three patients had previously been diagnosed with FMF. Most of our patients were patients who had no previous complaint of FMF. PFMS attack was seen as the first clinical manifestation of FMF in 11 patients. Two patients who did not respond to steroid treatment improved with anakinra treatment., Conclusions: PFMS is a rare condition of FMF disease. It may be the first clinical manifestation of FMF disease. Fever may not be seen in all patients. Clinicians should be aware of this situation., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: Our aim in this study was to reveal the clinical features of pediatric uveitis in the pandemic period and to compare it with the pre-pandemic era., Methods: This retrospective study included 187 children diagnosed with uveitis between the 11 th of March 2017 and the 11 th of March 2022. The patients were divided into two groups based on the date of diagnosis as follows; Group 1: Patients diagnosed in the pre-pandemic period (11 th March 2017-11 th March 2020); Group 2: Patients diagnosed in the pandemic period (12 th March 2020-11 th March 2022). Demographic data, characteristics of uveitis, underlying diseases, systemic treatment modalities, and complications were compared between the two groups., Results: A total of 187 (pre-pandemic 71, and pandemic 114) pediatric uveitis patients were recruited to the study. Fifty one percent (51%) of the patients were female. The number of patients diagnosed with uveitis increased approximately twice during the pandemic period compared to the pre-pandemic period. The frequency of anterior uveitis was found to be significantly higher in the pandemic period than in the pre-pandemic period (p = 0.037). It was mostly presented as symptomatic uveitis. There was a decrease in the diagnosis of JIA-related uveitis. ANA positivity increased in the pandemic period (p = 0.029). The response to treatment was better and the complication rate decreased in the pandemic period., Conclusion: The present study involved a large number of pediatric patients with uveitis. There are some differences in the characteristics of pediatric uveitis cases comparing the pandemic period and the pre-pandemic period. This increased frequency and changing clinical features of pediatric uveitis seems to be a result of COVID-19 infection.

Objective: COVID-19-associated pediatric vasculitis, other than Kawasaki disease (KD)-like vasculitis in multisystem inflammatory syndrome in children (MIS-C), is very rare. This study sought to analyze the characteristics, treatment, and outcomes in patients with COVID-19-associated pediatric vasculitis (excluding KD-like vasculitis in MIS-C)., Methods: The inclusion criteria were as follows: 1) age <18 years at vasculitis onset; 2) evidence of vasculitis; 3) evidence of SARS-CoV-2 exposure; and 4) ≤3 months between SARS-CoV-2 exposure and vasculitis onset. Patients with MIS-C were excluded. The features of the subset of patients in our cohort who had COVID-19-associated pediatric IgA vasculitis/Henoch Schönlein purpura (IgAV/HSP) were compared against a pre-pandemic cohort of pediatric IgAV/HSP patients., Results: Forty-one patients (median age 8.3 years; male to female ratio 1.3) were included from 14 centers and 6 countries. The most frequent vasculitis subtype was IgAV/HSP (n = 30). The median duration between SARS-CoV-2 exposure and vasculitis onset was 13 days. Involvement of the skin (92.7%) and of the gastrointestinal system (61%) were the most common manifestations of vasculitis. Most patients (68.3%) received glucocorticoids, and 14.6% also received additional immunosuppressive drugs. Remission was achieved in all patients. All of the patients with IgAV/HSP in our cohort had skin manifestations, while 18 (60%) had gastrointestinal involvement and 13 (43.3%) had renal involvement. When we compared the features of this subset of 30 patients to those of a pre-pandemic pediatric IgAV/HSP cohort (n = 159), the clinical characteristics of fever and renal involvement were more common in our COVID-19-associated pediatric IgAV/HSP cohort (fever, 30% versus 5%, respectively [P < 0.001]; renal involvement, 43.3% versus 17.6%, respectively [P = 0.002]). Recovery without treatment and complete recovery were each less frequent among our COVID-19-associated pediatric IgAV/HSP patients compared to the pre-pandemic pediatric IgAV/HSP cohort (recovery without treatment, 10% versus 39%, respectively [P = 0.002]; complete recovery, 86.7% versus 99.4%, respectively [P = 0.002])., Conclusion: This is the largest cohort of children with COVID-19-associated vasculitis (excluding MIS-C) studied to date. Our findings suggest that children with COVID-19-associated IgAV/HSP experience a more severe disease course compared to pediatric IgAV/HSP patients before the pandemic., (© 2022 American College of Rheumatology.)

Pediatric primary systemic vasculitides are a complex group of diseases. Vasculitis subgroups are mainly determined according to the size of the predominantly affected vessels. In patients with primary systemic vasculitis, the location of vascular involvement, the size of the vessels, the extent of vascular damage, and the underlying pathology determine the disease phenotype and severity. Cardiac involvement is rare in some pediatric vasculitis, such as IgA vasculitis and polyarteritis nodosa, while it is more common in some others like Kawasaki disease and Takayasu arteritis. On the other hand, chronic inflammation in the setting of systemic vasculitis forms a major cardiovascular risk factor. Accelerated atherosclerosis and the tendency to thrombosis are the main issues determining the cardiovascular risks in pediatric systemic vasculitis. Early diagnosis and treatment are essential in these patients to minimize morbidity and mortality. In this review, we aimed to raise physicians' awareness of cardiac involvement and cardiovascular risks in pediatric patients with primary systemic vasculitis., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objectives: Wide spread availability of safe and effective vaccines for COVID-19 in all countries is the best hope to end the COVID-19 pandemic. However, in developing countries, the hesitancy of the society about vaccination is an important problem in terms of public health. This study aimed to investigate the acceptability and tolerability of COVID-19 vaccines in the pediatric population diagnosed with rheumatic disease, as well as the attitudes toward these vaccines., Methods: This is an observational, cross sectional, single center study. Pediatric patients with at least one diagnosis of rheumatic disease were included in this study to investigate patient and family acceptability and safety of COVID-19 vaccines., Results: A total of 228 patients with rheumatic disease were included in this study. Ninety nine (43.4%) of the patients were juvenile idiopathic arthritis. One hundred and five (46%) of the patients were using biological agent treatment for their rheumatic disease, whereas 123 (54%) of the patients were not. No serious adverse effect related to the COVID-19 vaccine were observed in any of the patients. No disease activation was observed in any of them., Conclusion: There are only a few studies evaluating of the safety and disease flare of COVID-19 vaccines in children with rheumatic disease. Although this study has some limitations, such as the small sample size of patients with different diagnoses, it appears that there is no increase in COVID-19 vaccination-related harms in the patients with rheumatic disease.

Background: We aimed to compare the ten different scores (by Kobayashi, Egami, Harada, Formosa, Sano, Piram et al., Wu et al., Yang et al., Tan et al., and Kanai et al.) to assess their performance in predicting IVIG resistance in Turkish children., Methods: Complete and incomplete KD patients diagnosed with KD at Hacettepe University between June 2007 and September 2019 were evaluated retrospectively., Results: A total of 129 patients, 79 boys (61.2%), with a median age 36 (IQR 19.5-57.0) months were evaluated. Sixteen patients (12.4%) had IVIG resistance. Sensitivity was low for all the ten scores. Tan, Sano, and Egami predictive models had the highest specificity (97.3, 89.4, 86.7%, respectively). Almost all scoring systems distinguished the group of patients with low risk for IVIG resistance but could not differentiate IVIG-resistant patients. Multivariate analysis for the laboratory features showed that platelet count <300 × 10 9 /L and GGT serum levels were independent risk factors for IVIG resistance (OR: 3.896; 95% CI: 1.054-14.404; p = 0.042 and OR: 1.008; 95% CI: 1.001-1.015; p = 0.050)., Conclusions: The current scoring systems had a low sensitivity for predicting the risk for IVIG resistance in Turkish children. On the other hand, increased serum GGT levels and low platelet count were risk factors for predicting IVIG resistance., Impact: Intravenous immunoglobulin (IVIG) resistance may be observed in 10-20% of patients diagnosed with Kawasaki disease. Coronary artery involvement is more frequent in IVIG-resistant patients. It is important to predict the patients who might develop IVIG resistance to improve prognosis. The performance of the IVIG resistance predictive models in Kawasaki disease in our population is limited due to the low sensitivity., (© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Background: Kawasaki disease is an acute, febrile vasculitis of childhood that affects medium-sized arteries, predominantly the coronary arteries. It is a multisystem disease; therefore, it may present with non-cardiac findings of disease., Case Presentation: Here, we report the case of 7-year-old Turkish girl who presented with symptoms of fever, chest pain, and vomiting, who was diagnosed as having Kawasaki disease. We also present a literature review on pulmonary involvement due to Kawasaki disease., Conclusion: Pediatricians should consider the diagnosis of Kawasaki disease in the presence of pneumonia and pleural effusion that is nonresponsive to antibiotic therapy. This will prevent delay in diagnosis and the adverse consequences of the disease.

The aim of this study was to review the characteristics of patients with Kawasaki disease (KD) from Turkey and to assess the performance of the Kobayashi score (KS), Harada score (HS), Formosa score (FS), Egami score (ES) and other parameters in predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement (CAI) in the Turkish population. Patients who were diagnosed as being in the acute phase of KD at Hacettepe University Faculty of Medicine (Ankara, Turkey) between June 2007 and January 2016 reviewed retrospectively, and those between January 2016 and February 2018 reviewed prospectively, were included in this cohort study. A total of 100 patients with KD were included in this study. Statistical Package for Social Sciences for Windows 22.0 (SPSS Inc, Chicago, IL, USA) was used for statistical analysis. Eighty-five patients (85%) responded to IVIG treatment, whereas 15 (5 female, 10 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 31 (31%) (9 female; 22 male) patients. For predicting IVIG resistance, KS, ES, FS, and HS had sensitivity of 82.1%, 26.7%, 30.8%, 69.2% and specificity of 35.7%, 94%, 51.2%, 45.8%, respectively. For the association with CAI occurrence, the sensitivities were 17.2%, 3.3%, 35.7%, 70.4% and the specificities were 78.5%, 88.4%, 49.3%, 49.3% for the aforementioned scores, respectively. The multivariate analysis showed white blood cell (WBC) count [Odd's ratio (OR) 4.1; 95% confidence interval (CI) 1.26-13.23; p = 0.019] and hematocrit (OR 3.8; 95% CI 1.15-12.4; p = 0.028), as independent predictors of CAI while gamma-glutamyl transferase (GGT) level (OR 5.7; 95% CI 1.73-27.51; p = 0.018) was detected as the only independent predictor of IVIG resistance. This is the first study from Turkey in KD to evaluate the association of the scoring systems for IVIG resistance and CAI. The risk scoring systems in KD did not predict the risk for IVIG resistance and were not associated with CAI in Turkish population.