A1 Refereed original research article in a scientific journal
Clinical features, treatment and outcome of pediatric patients with severe cutaneous manifestations in IgA vasculitis: Multicenter international study
Authors: Sestan Mario, Kifer Nastasia, Held Martina, Frkovic Marijan, Jelusic Marija, Sozeri Betul, Demir Ferhat, Ulu Kadir,Silva Clovis A., Campos Reinan T. Batu Ezgi Deniz, Koker Oya, Sapina Matej, Josip Juraj Strossmayer Univ Osijek Med Fac Dept Paediat Osijek Croatia,Srsen Sasa, Gagro Alenka, Gagro Alenka, Fonseca Adriana Rodrigues, Rodrigues Marta, Rigante Donato, Filocamo Giovanni, Baldo Francesco, Hshin_Bekenstein Merav, Sorssmayer Jospi Jurai, Giani Teresa, Kataja Janne, Frkovic Marijan, Ruperto Nicolino, Ozen Seza, Jelusic Marija
Publisher: W B SAUNDERS CO-ELSEVIER INC
Publication year: 2023
Journal: Seminars in Arthritis and Rheumatism
Journal name in source: SEMINARS IN ARTHRITIS AND RHEUMATISM
Journal acronym: SEMIN ARTHRITIS RHEU
Article number: 152209
Volume: 61
Number of pages: 7
ISSN: 0049-0172
DOI: https://doi.org/10.1016/j.semarthrit.2023.152209
Web address : https://doi.org/10.1016/j.semarthrit.2023.152209
Objective: IgA vasculitis (IgAV) (formerly Henoch-Schönlein Purpura, HSP) rarely causes severe skin lesions in children. The purpose of the research was to determine whether severe skin manifestations were associated with a more severe disease course.
Methods: Severe cutaneous manifestations were defined as presence of hemorrhagic vesicles, bullae, ulcerations and/or necroses. Data were collected retrospectively from 12 international tertiary university medical centers.
Results: A total of 64 patients with the most severe skin changes in IgAV/HSP and median (Q1, Q3) age of 8.08 (5.08, 11.92) years at the disease onset were compared with 596 IgAV/HSP patients without these manfiestations and median (Q1, Q3) age of 6.33 (4.50, 8.92) years. The patients with severe cutaneous manifestations were older in comparison to other patients with IgAV/HSP (p<0.001), they developed nephritis more frequently (40.6% vs. 20.6%, p = 0.001) with worse outcome of renal disease (p = 0.001). This group of patients also had higher frequencies of severe gastrointestinal complications like hematochezia, massive bleeding and/or intussusception (29.3% vs. 14.8%, p<0.001). d-dimer concentrations were significantly higher in these patients (4.60 mg/L vs. 2.72 mg/L, p = 0.003) and they had more frequent need for treatment with systemic glucocorticoids (84.4% vs. 37.2%, p<0.001) in comparison with the control group. Further multivariate analysis showed that severe cutaneous changes were associated with higher risk of developing nephritis [OR=3.1 (95%CI 1.04-9.21), p = 0.042] and severe gastrointestinal complications [OR=3.65 (95%CI 1.08-12.37), p = 0.038].
Conclusion: Patients with IgAV/HSP and severe skin manifestations had a more severe clinical course and more frequently required glucocorticoids compared to classic IgAV/HSP patients.
