The Syndromes of Thrombotic Microangiopathy: A Critical Appraisal on Complement Dysregulation.
The Syndromes of Thrombotic Microangiopathy: A Critical Appraisal on Complement Dysregulation.
复制标题
DOI：
10.3390/jcm10143034
复制
发表时间：
2021-07-08
影响因子：
3.9
通讯作者：
van Paassen P
中科院分区：
文献类型：
--
作者：
Timmermans SAMEG;van Paassen P
血栓性微血管病（TMA）是一种罕见的，可能危及生命的疾病，可由一组异质性疾病引起，通常影响大脑和肾脏。应根据病因对TMA进行分类，以指示治疗目标。补体失调是TMA的一个重要原因，它定义了与共存疾病无关的病例，即原发性非典型溶血性尿毒综合征（HUS）。自从批准治疗性补体抑制以来，TMA的方法一直专注于识别原发性非典型HUS。然而，最近的进展表明，补体失调在被认为患有继发性非典型HUS的特定亚型患者中起着关键作用。这在同时存在高血压急症、妊娠和肾移植的患者中尤其如此，从而改变了疾病的范式。相比之下，补体失调在患有其他共存疾病的患者中是不常见的，例如细菌感染、药物使用、癌症和自身免疫以及其他疾病。在这篇综述中，我们进行了一个关键的评估补体失调和使用治疗性补体抑制与共存条件相关的TMA，并概述了一个务实的方法来诊断和治疗。对于未来的研究，我们主张长期补体介导的TMA，而不是传统的非典型HUS型分类。
Thrombotic microangiopathy (TMA) is a rare and potentially life-threatening condition that can be caused by a heterogeneous group of diseases, often affecting the brain and kidneys. TMAs should be classified according to etiology to indicate targets for treatment. Complement dysregulation is an important cause of TMA that defines cases not related to coexisting conditions, that is, primary atypical hemolytic uremic syndrome (HUS). Ever since the approval of therapeutic complement inhibition, the approach of TMA has focused on the recognition of primary atypical HUS. Recent advances, however, demonstrated the pivotal role of complement dysregulation in specific subtypes of patients considered to have secondary atypical HUS. This is particularly the case in patients presenting with coexisting hypertensive emergency, pregnancy, and kidney transplantation, shifting the paradigm of disease. In contrast, complement dysregulation is uncommon in patients with other coexisting conditions, such as bacterial infection, drug use, cancer, and autoimmunity, among other disorders. In this review, we performed a critical appraisal on complement dysregulation and the use of therapeutic complement inhibition in TMAs associated with coexisting conditions and outline a pragmatic approach to diagnosis and treatment. For future studies, we advocate the term complement-mediated TMA as opposed to the traditional atypical HUS-type classification.
登录
查看更多内容
DOI：
10.1007/s00467-016-3399-0
发表时间：
2017-05
期刊：
Pediatric nephrology (Berlin, Germany)
影响因子：
--
作者：
Beck BB;van Spronsen F;Diepstra A;Berger RM;Kömhoff M
通讯作者：
Kömhoff M
DOI：
10.4049/jimmunol.0804031
发表时间：
2009-06-01
期刊：
Journal of immunology (Baltimore, Md. : 1950)
影响因子：
--
作者：
Ferreira VP;Herbert AP;Cortés C;McKee KA;Blaum BS;Esswein ST;Uhrín D;Barlow PN;Pangburn MK;Kavanagh D
通讯作者：
Kavanagh D
影响因子：
19.6
作者：
Cavero, Teresa;Arjona, Emilia;Praga, Manuel
通讯作者：
Praga, Manuel
影响因子：
6.1
作者：
Ahlenstiel-Grunow, Thurid;Hachmeister, Svenja;Pape, Lars
通讯作者：
Pape, Lars
影响因子：
158.5
作者：
Eremina, Vera;Jefferson, J. Ashley;Quaggin, Susan E.
通讯作者：
Quaggin, Susan E.