Kang Hee Gyung, Hsu Danny, Kato Noritoshi, Kim Jin Seok, Okumi Masayoshi, Tseng Min-Hua, Tu Kun-Hua, Yap Desmond Yat Hin, Lim Wai H
Division of Pediatric Nephrology, Department of Pediatrics, Seoul National University Children's Hospital & Seoul National University College of Medicine, Seoul, South Korea.
Haematology Department, Liverpool Hospital, Liverpool, NSW, Australia.
Nephrology (Carlton). 2025 Sep;30(9):e70116. doi: 10.1111/nep.70116.
Complement-amplifying events/conditions associated with thrombotic microangiopathy (TMA) include pregnancy/postpartum period, severe hypertension, autoimmune diseases, drug exposures, infections and organ transplantation. Some of these 'triggers' may exist comorbidly with atypical haemolytic uraemic syndrome (aHUS; a complement-mediated form of TMA), unmask previously undiagnosed aHUS, or occur secondary to aHUS, thus creating a considerable diagnostic challenge. A major goal in patients presenting with TMA is to differentiate complement-mediated aHUS from other causes of TMA such that appropriate targeted treatment with complement 5 (C5) inhibitors can be initiated rapidly to avoid irreversible end-organ damage. To this end, nephrologists and haematologists from Australia, Hong Kong, Japan, Korea and Taiwan met virtually to discuss the management of TMA/aHUS in the presence of trigger conditions, focusing on the role of C5 inhibitors. To assist primary healthcare physicians and specialists from other disciplines in identifying and managing aHUS in the presence of triggers, the panel developed diagnostic and treatment algorithms as the main meeting output. Individual algorithms are presented for the settings of pregnancy, hypertension, autoimmune diseases, drug exposures, and kidney transplant. The algorithms combine clinical evidence with the panel's collective expertise to provide practical steps to differentiate aHUS and can be refined by local experts to reflect respective healthcare systems, approval and reimbursement procedures, resources and access to treatments for aHUS in any Asia-Pacific country.
与血栓性微血管病（TMA）相关的补体激活事件/情况包括妊娠/产后期、重度高血压、自身免疫性疾病、药物暴露、感染和器官移植。其中一些“触发因素”可能与非典型溶血性尿毒症综合征（aHUS，一种补体介导的TMA形式）合并存在，揭示先前未诊断出的aHUS，或继发于aHUS，从而带来相当大的诊断挑战。TMA患者的一个主要目标是将补体介导的aHUS与TMA的其他病因区分开来，以便能够迅速启动用补体5（C5）抑制剂进行的适当靶向治疗，避免不可逆的终末器官损伤。为此，来自澳大利亚、中国香港、日本、韩国和中国台湾的肾病学家和血液学家进行了线上会议，讨论在存在触发因素的情况下TMA/aHUS的管理，重点关注C5抑制剂的作用。为帮助基层医疗医生和其他学科的专家在存在触发因素的情况下识别和管理aHUS，该小组制定了诊断和治疗算法作为会议的主要成果。针对妊娠、高血压、自身免疫性疾病、药物暴露和肾移植等情况分别给出了算法。这些算法将临床证据与小组的集体专业知识相结合，提供区分aHUS的实际步骤，并且可由当地专家进行完善，以反映任何亚太国家各自的医疗体系、批准和报销程序、资源以及获得aHUS治疗的途径。
