Division of Nephrology, Department of Medicine, West China Hospital, Sichuan University, Chengdu, China.
West China School of Medicine, Sichuan University, Chengdu, China.
Kidney Blood Press Res. 2019;44(4):754-764. doi: 10.1159/000501459. Epub 2019 Aug 6.
Henoch-Schönlein purpura nephritis (HSPN) is a common vasculitis involving the kidneys, with a lower incidence in adults. Meanwhile, nephrotic syndrome (NS) can appear in HSPN. However, the clinicopathological features and renal outcome of adult-onset HSPN presenting with NS (NS-HSPN) have not been well clarified.
A total of 191 HSPN patients were prospectively analyzed and comparisons were made between NS-HSPN and non-NS-HSPN. Multivariate Cox regression analysis was carried out to find the unfavorable factors of renal outcome of NS-HSPN.
Among the 191 patients, 44 (23.0%) had NS-HSPN. Apart from edema and abdominal pain, patients with NS-HSPN tended to have lower levels of erythrocytes and hemoglobulin in blood as well as a greater number of erythrocytes in urine (p < 0.05). Mesangial proliferation was the most common pathological lesion in HSPN and the rates of crescent formation were significantly different, with 54.5% in NS-HSPN and 33.3% in non-NS-HSPN (p < 0.05). Notably, 18.2 and 4.8% of patients reached the composite endpoints in the NS-HSPN and non-NS-HSPN groups, respectively (p < 0.05), demonstrating that NS-HSPN patients were more likely to progress to end-stage renal disease and had a worse outcome. We also found that hypertension, estimated glomerular filtration rate (eGFR), cystatin, and tubular atrophy/interstitial fibrosis (HR > 1, p < 0.05) at onset were correlated with adverse outcome in NS-HSPN.
NS-HSPN had more severe clinicopathological manifestations and poorer prognosis. The adverse predictors of NS-HSPN principally depend on clinicopathological presentation rather than on different therapies, and hypertension, eGFR, cystatin, and tubular atrophy/interstitial fibrosis can serve as independent risk factors in NS-HSPN.
过敏性紫癜肾炎（HSPN）是一种常见的累及肾脏的小血管炎，成人发病率较低。同时，HSPN 可出现肾病综合征（NS）。然而，成人发病的 HSPN 合并 NS（NS-HSPN）的临床病理特征和肾脏预后尚未得到充分阐明。
对 191 例 HSPN 患者进行前瞻性分析，并对 NS-HSPN 与非 NS-HSPN 进行比较。采用多因素 Cox 回归分析寻找 NS-HSPN 肾脏预后不良的危险因素。
在 191 例患者中，44 例（23.0%）合并 NS-HSPN。除水肿和腹痛外，NS-HSPN 患者的血液红细胞和血红蛋白水平较低，尿液中红细胞较多（p<0.05）。系膜增生是 HSPN 最常见的病理病变，新月体形成的比例有显著差异，NS-HSPN 为 54.5%，非 NS-HSPN 为 33.3%（p<0.05）。值得注意的是，NS-HSPN 组和非 NS-HSPN 组分别有 18.2%和 4.8%的患者达到复合终点（p<0.05），表明 NS-HSPN 患者更易进展为终末期肾病，预后较差。我们还发现，发病时的高血压、估计肾小球滤过率（eGFR）、胱抑素和肾小管萎缩/间质纤维化（HR>1，p<0.05）与 NS-HSPN 的不良结局相关。
NS-HSPN 具有更严重的临床病理表现和更差的预后。NS-HSPN 的不良预测因素主要取决于临床病理表现，而不是不同的治疗方法，高血压、eGFR、胱抑素和肾小管萎缩/间质纤维化可作为 NS-HSPN 的独立危险因素。
