Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.
Department of Paediatrics, University of Split School of Medicine, University Hospital Centre Split, Split, Croatia.
Dermatology. 2022;238(2):340-346. doi: 10.1159/000516765. Epub 2021 Jun 7.
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.
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.
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.
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.
背景/目的：本研究旨在评估儿童 IgA 血管炎（IgAV）皮肤受累的发生率和严重程度及其与临床和生化参数的关系，以及发生 IgA 血管炎肾病（IgAVN）的风险，后者是 IgAV 患者长期发病的唯一原因，也是主要的预后因素。
本项全国性多中心回顾性研究纳入了 2009 年至 2019 年间五家克罗地亚三级医院就诊的 611 名年龄在 18 岁以下的 IgAV 患儿。通过对克罗地亚人群中 IgAV 患者的数据库进行系统分析，收集患者数据。
在 611 名儿童中，205 名（33.55%）下肢有紫癜，207 名（33.88%）皮疹扩展至躯干，149 名（24.39%）扩展至上肢，32 名（5.24%）皮疹泛发，而 15 名（2.47%）皮肤症状最严重：水疱、溃疡和坏死。130 名（21.28%）患儿发生 IgAVN，48 名（7.86%）患儿发生持续性 IgAVN（持续 3 个月以上）。多变量逻辑回归发现，存在溃疡和坏死（OR 3.20[95%CI 1.03-9.91]）、持续性紫癜（OR 2.89[95%CI 1.71-4.88]）和年龄较大（OR 1.16[95%CI 1.09-1.23]）是 IgAVN 的显著预测因素，而持续性紫癜（OR 20.11[95%CI 1.09-372.52]）、男性（OR 3.32[95%CI 1.13-9.80]）和年龄较大（OR 1.15[95%CI 1.00-1.30]）是持续性 IgAVN 的预测因素。在实验室参数中，血清尿素升高（OR 1.43[95%CI 1.03-2.00]）和活化部分凝血活酶时间缩短（OR 0.83[95%CI 0.74-0.93]）与持续性 IgAVN 风险增加显著相关。
随着 IgAV 皮肤表现的严重程度和持续时间的增加，发生 IgAVN 的风险增加，预后更差，更有可能需要更积极的治疗。
