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 clinical course of SARS-CoV-2 infection among children with rheumatic disease under biologic therapy: a retrospective and multicenter study.

The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.