Data_Sheet_1_Global Glomerulosclerosis and Segmental Glomerulosclerosis Could Serve as Effective Markers for Prognosis and Treatment of IgA Vasculitis With Nephritis.PDF

Background: This study was aimed at investigating the clinical significance and curative effect of global glomerulosclerosis (GS) and segmental glomerulosclerosis (S) in adult-onset IgA vasculitis with nephritis (IgAV-N) patients since there was no consensus pathological grading method for adult IgAV-N.
Methods: A total of 188 biopsy-proven IgAV-N patients were prospectively identified. Patients were separately assigned to GS0/GS1/GS2 group and S0/S1/S2 based on the scores of global glomerulosclerosis and segmental glomerulosclerosis (0% /0–15% />15%, respectively).
Results: GS0, GS1, and GS2 occurred in 56.4, 29.2, and 14.4% of the adult-onset IgAV-N, respectively. Patients in GS2 group tended to have the most serious renal deterioration and the highest levels of blood pressure. IgAV-N patients were also divided into S0 group (64.4%), S1 group (20.7%), and S2 group (14.9%), where no obvious differences in baseline data were noted. K–M curves indicated that GS2 group had the worst renal outcome (P = 0.05) while there seemed to be no significant differences between GS0 group and GS1 group. In addition, no remarkable differences in primary outcome were found among S0 group, S1 group, and S2 group though the prognosis of S2 group tended to be the worst. However, the prognosis of S0/S1 group was markedly better than that of S2 (P = 0.04). The discrimination of poor prognosis could be improved by adding the pathological indicators of global glomerulosclerosis and segmental glomerulosclerosis. Most importantly, immunosuppressive treatment might be a superior alternative in IgAV-N patients without sclerosis scores or with lower level of sclerosis scores. But addition of immunosuppression was not recommended in patients with higher sclerosis scores.
Conclusions: Global glomerulosclerosis and segmental sclerosis might be used for management and treatment of adult-onset IgAV-N.

背景：鉴于成人IgA血管炎伴肾炎（adult-onset IgA vasculitis with nephritis, IgAV-N）尚无统一的病理分级方法，本研究旨在探讨全球肾小球硬化（global glomerulosclerosis, GS）与节段性肾小球硬化（segmental glomerulosclerosis, S）在成人起病IgAV-N患者中的临床意义及疗效。
方法：本研究共前瞻性纳入188例经肾活检证实的IgAV-N患者，依据全球肾小球硬化与节段性肾小球硬化的评分分别分为GS0/GS1/GS2组与S0/S1/S2组（评分阈值分别为0%、0%~15%、>15%）。
结果：成人起病IgAV-N患者中，GS0、GS1及GS2组的占比分别为56.4%、29.2%与14.4%。GS2组患者往往存在最严重的肾功能恶化与最高的血压水平。此外，IgAV-N患者亦可分为S0组（64.4%）、S1组（20.7%）与S2组（14.9%），该三亚组的基线数据无显著差异。Kaplan-Meier（K-M）曲线显示，GS2组的肾脏预后最差（P=0.05），而GS0组与GS1组之间未观察到显著差异。另外，尽管S2组的预后往往最差，但S0、S1与S2组间的主要结局指标无显著差异；不过S0/S1组的预后显著优于S2组（P=0.04）。加入全球肾小球硬化与节段性肾小球硬化的病理指标可提升对不良预后的判别效能。最为关键的是，对于无硬化评分或硬化评分较低的IgAV-N患者，免疫抑制治疗或为更优选择；而对于硬化评分较高的患者，则不推荐加用免疫抑制治疗。
结论：全球肾小球硬化与节段性肾小球硬化可用于成人起病IgAV-N的临床管理与治疗。