The Challenge Of Treating Pulmonary Vasculitis In Behcet'S Disease: Two Pediatric Cases

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Comorbidities In Familial Mediterranean Fever

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The Helios (Hacettepe University Electronic Research Forms) Registry: Use Of Biologic Drugs In Autoinflammatory Diseases

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Validation Of A Novel Disease Classification In Hacettepe Takayasu Arteritis Cohort

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The Effect of Biologics in the Treatment of Multisystem Inflammatory Syndrome in Children (Mis-C): A Single-Center Propensity-Score-Matched Study

Multisystem inflammatory syndrome in children (MIS-C) is a serious condition characterized by excessive inflammation that can arise as a complication of SARS-CoV-2 infection in children. While our understanding of COVID-19 and MIS-C has been advancing, there is still uncertainty regarding the optimal treatment for MIS-C. In this study, we aimed to compare the clinical and laboratory outcomes of MIS-C patients treated with IVIG plus corticosteroids (CS) to those treated with IVIG plus CS and an additional biologic drug. We used the propensity score (PS)-matching method to assess the relationships between initial treatment and outcomes. The primary outcome was a left ventricular ejection fraction of less than 55% on day 2 or beyond and/or the requirement of inotrope support on day 2 or beyond. We included 79 MIS-C patients (median age 8.51 years, 33 boys) followed in our center. Among them, 50 children (25 in each group) were allocated to the PS-matched cohort sample. The primary outcome was observed in none of the patients in the IVIG and CS group, while it occurred in eight patients in the IVIG plus CS and biologic group (p = 0.04). MIS-C is a disorder that may progress rapidly and calls for extensive care. For definitive recommendations, further studies, including randomized control trials, are required

The characteristics of patients with COVID-19-associated pediatric vasculitis: an international, multicenter study

Objective: COVID-19 associated pediatric vasculitis other than Kawasaki disease (KD)-like vasculitis in multisystem inflammatory syndrome in children (MIS-C) is very rare. We aimed to analyze the characteristics, treatment and outcome in COVID-19-associated pediatric vasculitis (excluding KD-like vasculitis in MIS-C). Methods: The inclusion criteria were as follows: 1) <18 years at vasculitis onset; 2) evidence of vasculitis; 3) evidence of SARS-CoV-2 exposure; 4) ≤3 months between SARS-CoV-2 exposure and vasculitis onset. Patients with MIS-C were excluded. Results: Forty-one patients (median age 8.3 years; M/F=1.3) were included from 14 centers and six countries. The most frequent vasculitis subtype was IgA vasculitis/Henoch Schönlein purpura (IgAV/HSP) (n=30). The median duration between SARS-CoV-2 exposure and vasculitis onset was 13 days. Skin (92.7%) and gastrointestinal (61%) involvements were the most common manifestations of vasculitis. Most patients (68.3%) received corticosteroids; while 14.6% used additional immunosuppressive drugs. Remission was achieved in all. All IgAV/HSP patients had skin manifestations while 18 (60%) and 13 (43.3%) had gastrointestinal system and renal involvement, respectively. When we compared the features of these patients with those of a pre-pandemic pediatric IgAV/HSP cohort (n=159), fever (30% vs. 5%; p<0.001) and renal involvement (43.3% vs. 17.6%; p=0.002) were more common, while recovery without treatment (10% vs. 39%; p=0.002) and complete recovery (86.7% vs. 99.4%; p=0.002) were less frequent among COVID-19-associated IgAV/HSP patients. Conclusion: This is the largest cohort of children with COVID-19 associated vasculitis (excluding MIS-C). Our findings suggest a more severe disease course in COVID-19-associated pediatric IgAV/HSP patients compared to pre-pandemic patients. This article is protected by copyright. All rights reserved

The characteristics of patients with COVID-19-associated pediatric vasculitis: an international, multicenter study.

Objective: COVID-19 associated pediatric vasculitis other than Kawasaki disease (KD)-like vasculitis in multisystem inflammatory syndrome in children (MIS-C) is very rare. We aimed to analyze the characteristics, treatment and outcome in COVID-19-associated pediatric vasculitis (excluding KD-like vasculitis in MIS-C). Methods: The inclusion criteria were as follows: 1) <18 years at vasculitis onset; 2) evidence of vasculitis; 3) evidence of SARS-CoV-2 exposure; 4) ≤3 months between SARS-CoV-2 exposure and vasculitis onset. Patients with MIS-C were excluded. Results: Forty-one patients (median age 8.3 years; M/F=1.3) were included from 14 centers and six countries. The most frequent vasculitis subtype was IgA vasculitis/Henoch Schönlein purpura (IgAV/HSP) (n=30). The median duration between SARS-CoV-2 exposure and vasculitis onset was 13 days. Skin (92.7%) and gastrointestinal (61%) involvements were the most common manifestations of vasculitis. Most patients (68.3%) received corticosteroids; while 14.6% used additional immunosuppressive drugs. Remission was achieved in all. All IgAV/HSP patients had skin manifestations while 18 (60%) and 13 (43.3%) had gastrointestinal system and renal involvement, respectively. When we compared the features of these patients with those of a pre-pandemic pediatric IgAV/HSP cohort (n=159), fever (30% vs. 5%; p<0.001) and renal involvement (43.3% vs. 17.6%; p=0.002) were more common, while recovery without treatment (10% vs. 39%; p=0.002) and complete recovery (86.7% vs. 99.4%; p=0.002) were less frequent among COVID-19-associated IgAV/HSP patients. Conclusion: This is the largest cohort of children with COVID-19 associated vasculitis (excluding MIS-C). Our findings suggest a more severe disease course in COVID-19-associated pediatric IgAV/HSP patients compared to pre-pandemic patients. This article is protected by copyright. All rights reserved

Rhupus syndrome in children: A multi-center retrospective cohort study and literature review

Objective: In this study, we aimed to evaluate the characteristics of pediatric rhupus patients including all the related series in the literature. Methods: Thirty pediatric patients with rhupus syndrome from 12 different centers in Turkey were included in this study. The literature was also reviewed for pediatric patients with rhupus syndrome. Results: The most prominent phenotype of these 30 patients was juvenile idiopathic arthritis (JIA) (60%) at the disease onset and SLE (73.3%) at the last visit. Major SLE-related organ involvements were skin (80%), hematological system (53.3%), and kidney (23.3%). Arthritis was polyarticular (73.3%), asymmetric (66.7%), and erosive (53.3%) in most patients. Hydroxychloroquine (100%), glucocorticoids (86.7%), and mycophenolate mofetil (46.7%) were mostly used for SLE, while glucocorticoids (76.6%), methotrexate (73.3%), and nonsteroidal anti-inflammatory drugs (NSAIDs) (57.6%) were mainly preferred for JIA. Our literature search revealed 20 pediatric patients with rhupus syndrome (75% were RF positive). The most prominent phenotype was JIA (91.7%) at the disease onset and SLE (63.6%) at the last visit. Major SLE-related organ involvements were skin (66.7%), hematological system (58.3%), and kidney (58.3%). Arthritis was polyarticular (77.8%), asymmetric (63.6%), and erosive (83.3%) in most patients. Glucocorticoid (100%), hydroxychloroquine (76.9%), and azathioprine (46.2%) were mostly used for SLE, while methotrexate (76.9%) and NSAIDs (46.2%) were mainly preferred for the JIA phenotype. Conclusion: Our study is the largest cohort in the literature evaluating pediatric rhupus cases. Most of the pediatric patients had polyarticular, asymmetric, and erosive arthritis, as well as organ involvements associated with SLE, including the skin, hematological system, and kidney

Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis.

ObjectiveProspective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR.MethodsConsenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants.ResultsOne hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event.ConclusionThe CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER