Servicio de Nefrología, Hospital 12 de Octubre, Madrid, España.
Servicio de Anatomía Patológica, Hospital 12 de Octubre, Madrid, España.
Med Clin (Barc). 2018 Oct 23;151(8):329-335. doi: 10.1016/j.medcli.2018.02.016. Epub 2018 Apr 24.
Haemolytic uremic syndrome (HUS) is characterised by microangiopathic haemolytic anaemia with acute kidney injury. It is currently classified into two main categories: Shiga-toxin producing E. coli-hemolytic uremic syndrome (STEC-HUS) and atypical haemolytic uremic syndrome (aHUS). Endothelial cell damage is the common pathway in HUS to developing thrombotic microangiopathy. Atypical HUS includes primary, secondary and aHUS due to metabolic diseases. In the majority of aHUS cases, hyperactivity of the alternative complement pathway plays a central role. Therefore, treatment is based on complement inhibitors like eculizumab, a drug that has revolutionised the natural history of the disease. Relapses are frequent after kidney transplant and thus confer a poor prognosis.
溶血性尿毒症综合征 (HUS) 的特征是伴有急性肾损伤的微血管病性溶血性贫血。目前将其分为两类：产志贺样毒素大肠杆菌溶血性尿毒症综合征 (STEC-HUS) 和非典型溶血性尿毒症综合征 (aHUS)。内皮细胞损伤是 HUS 发展为血栓性微血管病的共同途径。非典型 HUS 包括原发性、继发性和由代谢疾病引起的 aHUS。在大多数 aHUS 病例中，补体替代途径的过度活跃起着核心作用。因此，治疗基于补体抑制剂，如依库珠单抗，该药彻底改变了疾病的自然病程。肾移植后常复发，因此预后不良。
