Department of Nephrology (Key Laboratory of Management of Kidney Disease in Zhejiang Province), Guangxing Hospital Affiliated to Zhejiang Chinese Medical University (Hangzhou Hospital of Traditional Chinese Medicine), Hangzhou 310007, China.
Ann Palliat Med. 2020 May;9(3):795-804. doi: 10.21037/apm.2020.04.04. Epub 2020 Apr 26.
Clinical manifestations and histological lesions of IgA nephropathy and Henoch-Schönlein purpura nephritis (HSPN) are different, but related, and are also correlated with the renal outcomes. This study aimed to compare the features of immunoglobulin A nephropathy (IgAN) and HSPN in adult patients with diffuse endocapillary proliferation (DEP) lesions aiming to clarify the differences and relationships in the clinicopathological findings and outcome.
Twelve patients with DEP-IgAN and 10 patients with DEP-HSPN were enrolled. Twenty four patients with IgAN (NDEP-IgAN) and matched 20 patients with HSPN (NDEP-HSPN) were enrolled at the same ratio (1:2). The clinicopathological features, clinical efficacy, and renal outcomes were analyzed in the four groups.
DEP patients with IgAN or HSPN had worse clinical manifestations (more severe proteinuria, lower serum ALB, higher incidence of gross hematuria). The proteinuria in the DEP-HSPN group was more severe than in the DEP-IgAN group. There was no significant difference in the serum creatinine among four groups. The incidence of endothelial swelling was significantly higher in the DEP-HSPN group than in the NDEP-HSPN group and DEP-IgAN group. The S1 score of Oxford classification was more common in the DEP-IgAN group than in the DEP-HSPN. None in the DEP-IgAN group reached endpoint events during the follow-up period, while the renal outcomes were significantly poorer in the DEP-HSPN group than in the DEP-IgAN and NDEP-HSPN groups. No significant difference was observed in the cumulative renal survival among four groups (χ 2 =7.264, P=0.064), but patients in the DEP-HSPN group had markedly lower renal cumulative survival rate as compared to the NDEP-HSPN group (χ 2 =4.875, P=0.027).
The DEP is significantly associated with more severe proteinuria and hematuria regardless the IgAN and HSPN. Among DEP patients, patients with HSPN have poor therapeutic efficacy and renal outcomes, even under active immunosuppressive therapy, as compared to those with IgAN.
IgA 肾病和过敏性紫癜性肾炎（HSPN）的临床表现和组织学病变不同，但相关，与肾脏结局相关。本研究旨在比较弥漫性内皮细胞增生（DEP）病变成人患者中 IgA 肾病（IgAN）和 HSPN 的特征，旨在阐明临床病理表现和结果的差异和关系。
纳入 12 例 DEP-IgAN 患者和 10 例 DEP-HSPN 患者。以 1:2 的比例纳入 24 例 IgAN（NDEP-IgAN）患者和 20 例 HSPN（NDEP-HSPN）患者。分析四组患者的临床病理特征、临床疗效和肾脏结局。
DEP 患者无论患 IgAN 还是 HSPN，临床表现均更差（蛋白尿更严重，血清 ALB 更低，肉眼血尿发生率更高）。DEP-HSPN 组的蛋白尿比 DEP-IgAN 组更严重。四组患者血清肌酐无显著差异。DEP-HSPN 组内皮细胞肿胀发生率明显高于 NDEP-HSPN 组和 DEP-IgAN 组。Oxford 分类的 S1 评分在 DEP-IgAN 组更常见。DEP-IgAN 组在随访期间均未达到终点事件，而 DEP-HSPN 组的肾脏结局明显差于 DEP-IgAN 组和 NDEP-HSPN 组。四组患者的累积肾脏生存率无显著差异（χ 2 =7.264，P=0.064），但与 NDEP-HSPN 组相比，DEP-HSPN 组患者的累积肾脏生存率明显较低（χ 2 =4.875，P=0.027）。
DEP 与更严重的蛋白尿和血尿显著相关，无论 IgAN 还是 HSPN 都是如此。在 DEP 患者中，与 IgAN 患者相比，即使在积极的免疫抑制治疗下，HSPN 患者的治疗效果和肾脏结局也较差。
