Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome
Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome
复制标题
DOI：
10.1016/j.kint.2019.05.014
复制
发表时间：
2019-10-01
影响因子：
19.6
通讯作者：
Praga, Manuel
中科院分区：
文献类型：
--
作者：
Cavero, Teresa;Arjona, Emilia;Praga, Manuel
恶性高血压是继发性血栓性微血管病的原因之一，但在高血压诱导的血栓性微血管病患者中已报告补体基因的致病性突变。在这里，我们调查了55名原发性非典型溶血性尿毒综合征（阿胡斯）患者高血压的频率和严重程度。对所有患者进行遗传学分析，并对所有2级和3级高血压患者进行眼底镜检查。110例非阿胡斯引起的恶性高血压患者作为对照组。36例阿胡斯患者表现为2级或3级高血压，19例眼底镜检查显示恶性高血压。19例患者（恶性高血压患者中占37%）发现补体遗传异常。46名患者接受了血浆置换术，26名患者接受了依库珠单抗治疗。血浆置换术后的肾脏和血液学应答（24%）显著低于依库珠单抗（81%）。接受依库珠单抗治疗的患者（1年、3年和5年时为85%）的肾存活率显著高于未接受该治疗的患者（分别为54%、46%和41%）。对依库珠单抗的应答与高血压严重程度和补体遗传异常的存在无关。在其他疾病引起的恶性高血压患者中，血栓性微血管病的患病率非常低（5%）。因此，重度和恶性高血压在患有阿胡斯的患者中是常见的，并且与血浆置换相比，依库珠单抗治疗导致更高的肾存活率。然而，血栓性微血管病在阿胡斯以外的疾病引起的恶性高血压患者中并不常见。
Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. A cohort of 110 patients with malignant hypertension caused by diseases other than aHUS served as control. Thirty-six patients with aHUS presented Grade 2 or Grade 3 hypertension and funduscopic examination showed malignant hypertension in 19. Genetic abnormalities in complement were found in 19 patients (37% among patients with malignant hypertension). Plasmapheresis was performed in 46 patients and 26 received eculizumab. Renal and hematological responses were significantly lower after plasmapheresis (24%) than after eculizumab (81%). Renal survival was significantly higher in patients treated with eculizumab (85% at one, three and five years) compared to patients who did not receive this treatment (54%, 46% and 41%), respectively. Response to eculizumab was independent of hypertension severity and the presence of complement genetic abnormalities. Among patients with malignant hypertension caused by other diseases the prevalence of thrombotic microangiopathy was very low (5%). Thus, severe and malignant hypertension are common among patients with aHUS and eculizumab treatment leads to a higher renal survival when compared to plasmapheresis. However, thrombotic microangiopathy is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.