PDF(Pubmed)
Abstract：
UNASSIGNED: In pregnancy-related atypical hemolytic uremic syndrome (p-aHUS), transferring recommendations for treatment decisions from nonpregnant cohorts with thrombotic microangiopathy (TMA) is difficult. Although potential causes of p-aHUS may be unrelated to inherent complement defects, peripartal complications such as postpartum hemorrhage (PPH) or (pre)eclampsia or Hemolysis, Elevated Liver enzymes and Low Platelets (HELLP) syndrome may be unrecognized drivers of complement activation.
UNASSIGNED: To evaluate diagnostic and therapeutic decisions in the practical real-life setting, we conducted an analysis of a cohort of 40 patients from 3 German academic hospitals with a diagnosis of p-aHUS, stratified by the presence (n = 25) or absence (n = 15) of PPH.
UNASSIGNED: Histological signs of TMA were observed in 84.2% of all patients (100% vs. 72.7% in patients without or with PPH, respectively). Patients without PPH had a higher likelihood (20% vs. 0%) of pathogenic genetic abnormalities in the complement system although notably less than in other published cohorts. Four of 5 patients with observed renal cortical necrosis (RCN) after PPH received complement inhibition and experienced partially recovered kidney function. Patients on complement inhibition with or without PPH had an increased need for kidney replacement therapy (KRT) and plasma exchange (PEX). Because renal recovery was comparable among all patients treated with complement inhibition, a potential beneficial effect in this group of pregnancy-associated TMAs and p-aHUS is presumed.
UNASSIGNED: Based on our findings, we suggest a pragmatic approach toward limited and short-term anticomplement therapy for patients with a clinical diagnosis of p-aHUS, which should be stopped once causes of TMA other than genetic complement abnormalities emerge.
UNASSIGNED: To evaluate diagnostic and therapeutic decisions in the practical real-life setting, we conducted an analysis of a cohort of 40 patients from 3 German academic hospitals with a diagnosis of p-aHUS, stratified by the presence (n = 25) or absence (n = 15) of PPH.
UNASSIGNED: Histological signs of TMA were observed in 84.2% of all patients (100% vs. 72.7% in patients without or with PPH, respectively). Patients without PPH had a higher likelihood (20% vs. 0%) of pathogenic genetic abnormalities in the complement system although notably less than in other published cohorts. Four of 5 patients with observed renal cortical necrosis (RCN) after PPH received complement inhibition and experienced partially recovered kidney function. Patients on complement inhibition with or without PPH had an increased need for kidney replacement therapy (KRT) and plasma exchange (PEX). Because renal recovery was comparable among all patients treated with complement inhibition, a potential beneficial effect in this group of pregnancy-associated TMAs and p-aHUS is presumed.
UNASSIGNED: Based on our findings, we suggest a pragmatic approach toward limited and short-term anticomplement therapy for patients with a clinical diagnosis of p-aHUS, which should be stopped once causes of TMA other than genetic complement abnormalities emerge.
摘要：
■在妊娠相关的非典型溶血性尿毒综合征（p-aHUS）中，从患有血栓性微血管病(TMA)的非妊娠队列中转移治疗决策建议是困难的.尽管p-aHUS的潜在原因可能与固有补体缺陷无关，围产期并发症，如产后出血（PPH）或（先兆）子痫或溶血，肝酶升高和低血小板（HELLP）综合征可能是补体激活的未识别驱动因素。
■为了评估实际现实生活中的诊断和治疗决策，我们对来自3家德国学术医院诊断为p-aHUS的40例患者进行了分析，通过存在(n=25)或不存在(n=15)PPH进行分层。
■在所有患者的84.2％中观察到TMA的组织学征象（100％vs.无PPH或有PPH的患者占72.7%，分别)。没有PPH的患者有更高的可能性(20%vs.0％）的补体系统中的致病性遗传异常，尽管明显少于其他已发表的队列。5例PPH后观察到肾皮质坏死（RCN）的患者中有4例接受了补体抑制，肾功能部分恢复。有或没有PPH的补体抑制患者对肾脏替代疗法（KRT）和血浆置换（PEX）的需求增加。因为所有接受补体抑制治疗的患者的肾脏恢复情况相当，推测在这组与妊娠相关的TMAs和p-aHUS中有潜在的有益作用.
■根据我们的发现，我们建议对临床诊断为p-aHUS的患者进行有限和短期抗补体治疗的务实方法，一旦出现遗传补体异常以外的TMA原因，应停止。
■为了评估实际现实生活中的诊断和治疗决策，我们对来自3家德国学术医院诊断为p-aHUS的40例患者进行了分析，通过存在(n=25)或不存在(n=15)PPH进行分层。
■在所有患者的84.2％中观察到TMA的组织学征象（100％vs.无PPH或有PPH的患者占72.7%，分别)。没有PPH的患者有更高的可能性(20%vs.0％）的补体系统中的致病性遗传异常，尽管明显少于其他已发表的队列。5例PPH后观察到肾皮质坏死（RCN）的患者中有4例接受了补体抑制，肾功能部分恢复。有或没有PPH的补体抑制患者对肾脏替代疗法（KRT）和血浆置换（PEX）的需求增加。因为所有接受补体抑制治疗的患者的肾脏恢复情况相当，推测在这组与妊娠相关的TMAs和p-aHUS中有潜在的有益作用.
■根据我们的发现，我们建议对临床诊断为p-aHUS的患者进行有限和短期抗补体治疗的务实方法，一旦出现遗传补体异常以外的TMA原因，应停止。
