Nishida Reimi, Kaneko Shuzo, Usui Joichi, Kawamura Tetsuya, Tsunoda Ryoya, Tawara Takashi, Fujita Akiko, Nagai Kei, Kai Hirayasu, Morito Naoki, Saito Chie, Yamagata Kunihiro
Department of Nephrology and Pathology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
Ther Apher Dial. 2019 Jun;23(3):253-260. doi: 10.1111/1744-9987.12830.
Plasma exchange (PEX) can be an effective treatment in anti-neutrophil cytoplasmic antibody-associated vasculitis with severe renal damage; however, it is still controversial. Among cases of newly diagnosed AAV with rapidly progressive glomerulonephritis at our department from 2008 onward, 11 patients who received PEX (seven cases for severe renal damage [R-PEX] and four cases for lung hemorrhage [L-PEX]) were retrospectively analyzed. All cases of R-PEX were dependent on hemodialysis at the beginning of PEX and all received seven sessions of PEX (50 mL/kg or 1.3 plasma volume per exchange) within 2 weeks. All cases became dialysis-independent within 8 weeks, with 3- and 12-month cumulative renal survival rates of 100% and 80%, respectively. All cases of L-PEX retained their renal function. In rapidly developing, newly dialysis-dependent antibody-associated vasculitis with rapidly progressive glomerulonephritis patients with normal renal function before disease onset, standard PEX can be expected to induce sufficient renal recovery to establish dialysis independence.
血浆置换（PEX）对于抗中性粒细胞胞浆抗体相关性血管炎合并严重肾损害可能是一种有效的治疗方法；然而，其仍存在争议。对2008年起在我科新诊断的伴有快速进展性肾小球肾炎的抗中性粒细胞胞浆抗体相关性血管炎病例中，11例接受血浆置换的患者（7例因严重肾损害接受血浆置换[R-PEX]，4例因肺出血接受血浆置换[L-PEX]）进行回顾性分析。所有R-PEX病例在血浆置换开始时均依赖血液透析，且均在2周内接受了7次血浆置换（每次50 mL/kg或每次置换1.3个血浆量）。所有病例在8周内均不再依赖透析，3个月和12个月的累积肾脏生存率分别为100%和80%。所有L-PEX病例均保留了肾功能。在疾病发作前肾功能正常的快速进展、新出现透析依赖的抗体相关性血管炎合并快速进展性肾小球肾炎患者中，标准血浆置换有望诱导足够的肾脏恢复以实现不再依赖透析。
