Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, SP, Brazil.
Pediatric Nephrology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, SP, Brazil.
Clin Rheumatol. 2018 May;37(5):1319-1324. doi: 10.1007/s10067-017-3972-3. Epub 2018 Jan 13.
The objective of this study was to evaluate prevalence, initial risk factors, and outcomes in Henoch-Schönlein purpura nephritis (HSPN) patients in Latin America. Two hundred ninety-six patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements, and treatments in the first 3 months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non-nephrotic proteinuria, nephrotic proteinuria, and acute/chronic kidney injury were also systematically evaluated at 1, 5, 10, and 15 years after diagnosis. HSPN was evidenced in 139/296 (47%) in the first 3 months. The median age at diagnosis was significantly higher in HSPN patients compared without renal involvement [6.6 (1.5-17.7) vs. 5.7 (0.9-13.5) years, p = 0.022]. The frequencies of persistent purpura (31 vs. 10%, p < 0.0001), recurrent abdominal pain (16 vs. 7%, p = 0.011), gastrointestinal bleeding (25 vs. 10%, p < 0.0001), and corticosteroid use (54 vs. 41%, p = 0.023) were significantly higher in the former group. Logistic regression demonstrated that the independent variables associated with HSNP were persistent purpura (OR = 3.601; 95% CI (1.605-8.079); p = 0.002) and gastrointestinal bleeding (OR = 2.991; 95% CI (1.245-7.183); p = 0.014). Further analysis of patients without HSPN in the first 3 months revealed that 29/118 (25%) had persistent non-nephrotic proteinuria and/or hematuria in 1 year, 19/61 (31%) in 5 years, 6/17 (35%) in 10 years and 4/6 (67%) in 15 years after diagnosis. None of them had chronic kidney injury or were submitted to renal replacement therapy. The present study observed HSPN in almost one half of patients in the first months of disease, and HSPN was associated with persistent purpura and gastrointestinal bleeding. One fourth of patients had nephritis only evidenced during follow-up without severe renal manifestations.
本研究旨在评估拉丁美洲过敏性紫癜肾炎（HSPN）患者的患病率、首发危险因素和结局。通过人口统计学数据、临床/实验室检查和诊断后 3 个月内的治疗情况，对 296 例患者（经 EULAR/PRINTO/PRES HSP 标准验证）进行评估。这些患者在拉丁美洲的一家三级中心接受随访，并根据是否存在肾炎分为两组。在诊断后 1、5、10 和 15 年，还对持续性非肾病性蛋白尿、肾病性蛋白尿和急性/慢性肾损伤进行了系统评估。在诊断后 3 个月内，有 139/296（47%）例患者出现 HSPN。HSPN 患者的中位年龄明显高于无肾受累患者[6.6（1.5-17.7）岁 vs. 5.7（0.9-13.5）岁，p=0.022]。持续性紫癜（31% vs. 10%，p<0.0001）、反复腹痛（16% vs. 7%，p=0.011）、胃肠道出血（25% vs. 10%，p<0.0001）和皮质激素使用率（54% vs. 41%，p=0.023）在前者中显著更高。Logistic 回归分析表明，与 HSPN 相关的独立变量为持续性紫癜（OR=3.601；95%CI（1.605-8.079）；p=0.002）和胃肠道出血（OR=2.991；95%CI（1.245-7.183）；p=0.014）。进一步分析诊断后 3 个月内无 HSPN 的 118 例患者发现，1 年后有 29/118（25%）例患者持续存在非肾病性蛋白尿和/或血尿，5 年后有 19/61（31%）例患者，10 年后有 6/17（35%）例患者，15 年后有 4/6（67%）例患者。他们均无慢性肾脏损伤或接受肾脏替代治疗。本研究观察到，在疾病的最初几个月内，几乎有一半的患者出现 HSPN，且 HSPN 与持续性紫癜和胃肠道出血相关。四分之一的患者仅在随访中发现肾炎，且无严重肾脏表现。
