Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children

Mario Sestan; Sasa Srsen; Nastasia Kifer; Matej Sapina; Mateja Batnozic Varga; Aleksandar Ovuka; Martina Held; Ana Kozmar; Marijan Frkovic; Gordana Laskarin; Alenka Gagro; Marija Jelusic

doi:10.1159/000516765

Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children

Dermatology. 2022;238(2):340-346. doi: 10.1159/000516765. Epub 2021 Jun 7.

Authors

Affiliations

¹ Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.
² Department of Paediatrics, University of Split School of Medicine, University Hospital Centre Split, Split, Croatia.
³ Department of Paediatrics, Josip Juraj Strossmayer University of Osijek, Medical Faculty and Faculty of Dental Medicine and Health Osijek, University Hospital Centre Osijek, Osijek, Croatia.
⁴ Department of Paediatrics, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, University Hospital Centre Osijek, Osijek, Croatia.
⁵ Department of Paediatrics, University of Rijeka, Faculty of Medicine, University Hospital Centre Rijeka, Rijeka, Croatia.
⁶ Clinical Department of Laboratory Diagnostics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.
⁷ Faculty of Medicine, University of Rijeka, Rijeka, Croatia.
⁸ Department of Paediatrics, Children's Hospital Zagreb, Zagreb, Croatia.
⁹ Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Osijek, Croatia.

PMID: 34098552
DOI: 10.1159/000516765

Abstract

Background/objective: The purpose of this study was to evaluate the prevalence and severity of skin involvement in children with IgA vasculitis (IgAV) and its relationship with clinical and biochemical parameters and the risk of developing IgA vasculitis nephritis (IgAVN), the only cause of long-term morbidity and the main prognostic factor in IgAV patients.

Methods: This national multicenter retrospective study included 611 patients under the age of 18 years with IgAV referred to five Croatian tertiary hospitals between 2009 and 2019. Patient data were collected from a database with systematic analysis of IgAV patients in the Croatian population.

Results: Among the 611 children, 205 (33.55%) had purpura on the lower extremities, in 207 (33.88%) the rash extended on the trunk, in 149 (24.39%) it extended to the upper extremities, in 32 (5.24%) the rash was generalized, while 15 (2.47%) had the most severe skin symptoms: bullae, ulcerations, and necroses. IgAVN developed in 130 (21.28%) and persistent IgAVN (present for >3 months) in 48 (7.86%) children. Multivariate logistic regression found that presence of ulcerations and necroses (OR 3.20 [95% CI 1.03-9.91]), persistent purpura (OR 2.89 [95% CI 1.71-4.88]), and higher age (OR 1.16 [95% CI 1.09-1.23]) were significant predictors of IgAVN, whereas persistent purpura (OR 20.11 [95% CI 1.09-372.52]), male sex (OR 3.32 [95% CI 1.13-9.80]), and higher age (OR 1.15 [95% CI 1.00-1.30]) were predictors of persistent IgAVN. Among the laboratory parameters, higher serum urea (OR 1.43 [95% CI 1.03-2.00]) and reduction in activated partial thromboplastin time (OR 0.83 [95% CI 0.74-0.93]) were shown to have a significant impact on increasing the risk of persistent IgAVN.

Conclusion: With increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse, with a greater likelihood to need more aggressive treatment.

Keywords: Cutaneous manifestations; IgA vasculitis; IgA vasculitis nephritis; Risk factors.

Publication types

Multicenter Study

MeSH terms

Adolescent
Child
Humans
IgA Vasculitis* / complications
IgA Vasculitis* / epidemiology
Immunoglobulin A
Male
Nephritis*
Retrospective Studies
Vasculitis* / epidemiology
Vasculitis* / etiology

Substances

Immunoglobulin A