Division of Nephrology, Texas Tech Health Sciences Center El Paso, El Paso, TX 79905, USA.
Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Int J Mol Sci. 2023 Feb 16;24(4):3977. doi: 10.3390/ijms24043977.
Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor prognosis despite aggressive immunosuppressive therapy. The utility of plasmapheresis/plasma exchange (PLEX) for IgAN/HSP is not well established. This systematic review aims to assess the efficacy of PLEX for IgAN and HSP patients with RPGN. A literature search was conducted using MEDLINE, EMBASE, and through Cochrane Database from inception through September 2022. Studies that reported outcomes of PLEX in IgAN or HSP patients with RPGN were enrolled. The protocol for this systematic review is registered with PROSPERO (no. CRD42022356411). The researchers systematically reviewed 38 articles (29 case reports and 9 case series articles) with a total of 102 RPGN patients (64 (62.8%) had IgAN and 38 (37.2%) had HSP). The mean age was 25 years and 69% were males. There was no specific PLEX regimen utilized in these studies, but most patients received at least 3 PLEX sessions that were titrated based on the patient's response/kidney recovery. The number of PLEX sessions ranged from 3 to 18, and patients additionally received steroids and immunosuppressive treatment (61.6% of patients received cyclophosphamide). Follow-up time ranged from 1 to 120 months, with the majority being followed for at least 2 months after PLEX. Among IgAN patients treated with PLEX, 42.1% ( = 27/64) achieved remission; 20.3% ( = 13/64) achieved complete remission (CR) and 18.7% ( = 12/64) partial remission (PR). 60.9% ( = 39/64) progressed to end-stage kidney disease (ESKD). Among HSP patients treated with PLEX, 76.3% (n = 29/38) achieved remission; of these, 68.4% ( = 26/38) achieved CR and 7.8% achieved ( = 3/38) PR. 23.6% ( = 9/38) progressed to ESKD. Among kidney transplant patients, 20% (n = 1/5) achieved remission and 80% ( = 4/5) progressed to ESKD. Adjunctive plasmapheresis/plasma exchange with immunosuppressive therapy showed benefits in some HSP patients with RPGN and possible benefits in IgAN patients with RPGN. Future prospective, multi-center, randomized clinical studies are needed to corroborate this systematic review's findings.
患有 IgA 肾病（IgAN），包括过敏性紫癜性肾炎（HSP），并伴有急进性肾小球肾炎（RPGN）的患者，尽管接受了强化免疫抑制治疗，预后仍不佳。血浆置换（PLEX）在 IgAN/HSP 中的作用尚未得到充分证实。本系统评价旨在评估 PLEX 治疗 IgAN 和 HSP 合并 RPGN 患者的疗效。研究人员使用 MEDLINE、EMBASE 和 Cochrane 数据库进行了文献检索，检索时间从建库至 2022 年 9 月。纳入了报告 PLEX 治疗 IgAN 或 HSP 合并 RPGN 患者结局的研究。本系统评价的方案已在 PROSPERO（编号：CRD42022356411）上注册。研究人员系统地回顾了 38 篇文章（29 篇病例报告和 9 篇病例系列文章），共纳入 102 例 RPGN 患者（64 例（62.8%）为 IgAN，38 例（37.2%）为 HSP）。平均年龄为 25 岁，69%为男性。这些研究中没有使用特定的 PLEX 方案，但大多数患者至少接受了 3 次 PLEX 治疗，根据患者的反应/肾脏恢复情况进行滴定。PLEX 治疗的次数从 3 次到 18 次不等，患者还接受了类固醇和免疫抑制治疗（61.6%的患者接受环磷酰胺治疗）。随访时间从 1 到 120 个月不等，大多数患者在 PLEX 治疗后至少随访 2 个月。在接受 PLEX 治疗的 IgAN 患者中，42.1%（=27/64）达到缓解；20.3%（=13/64）达到完全缓解（CR），18.7%（=12/64）达到部分缓解（PR）。60.9%（=39/64）进展为终末期肾病（ESKD）。在接受 PLEX 治疗的 HSP 患者中，76.3%（n=29/38）达到缓解；其中，68.4%（=26/38）达到 CR，7.8%达到（=3/38）PR。23.6%（=9/38）进展为 ESKD。在肾移植患者中，20%（n=1/5）达到缓解，80%（=4/5）进展为 ESKD。免疫抑制治疗联合辅助性血浆置换/血浆交换在某些 HSP 合并 RPGN 患者中显示出获益，在 IgAN 合并 RPGN 患者中可能也有获益。需要进一步开展前瞻性、多中心、随机临床试验来证实本系统评价的研究结果。
