Abbas Fedaey, El Kossi Mohsen, Kim Jon Jin, Shaheen Ihab Sakr, Sharma Ajay, Halawa Ahmed
Nephrology Department, Jaber El Ahmed Military Hospital, Safat 13005, Kuwait.
Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom.
World J Transplant. 2018 Oct 22;8(6):203-219. doi: 10.5500/wjt.v8.i6.203.
For decades, kidney diseases related to inappropriate complement activity, such as atypical hemolytic uremic syndrome and C3 glomerulopathy (a subtype of membranoproliferative glomerulonephritis), have mostly been complicated by worsened prognoses and rapid progression to end-stage renal failure. Alternative complement pathway dysregulation, whether congenital or acquired, is well-recognized as the main driver of the disease process in these patients. The list of triggers include: surgery, infection, immunologic factors, pregnancy and medications. The advent of complement activation blockade, however, revolutionized the clinical course and outcome of these diseases, rendering transplantation a viable option for patients who were previously considered as non-transplantable cases. Several less-costly therapeutic lines and likely better efficacy and safety profiles are currently underway. In view of the challenging nature of diagnosing these diseases and the long-term cost implications, a multidisciplinary approach including the nephrologist, renal pathologist and the genetic laboratory is required to help improve overall care of these patients and draw the optimum therapeutic plan.
几十年来，与补体活性异常相关的肾脏疾病，如非典型溶血性尿毒症综合征和C3肾小球病（膜增生性肾小球肾炎的一种亚型），大多预后较差，且会迅速进展为终末期肾衰竭。补体替代途径失调，无论是先天性的还是后天获得性的，都被公认为这些患者疾病进程的主要驱动因素。引发因素包括：手术、感染、免疫因素、妊娠和药物。然而，补体激活阻断疗法的出现彻底改变了这些疾病的临床病程和结局，使移植成为以前被视为不可移植病例的患者的可行选择。目前正在开展几种成本较低的治疗方案，其疗效和安全性可能更好。鉴于诊断这些疾病具有挑战性以及长期成本影响，需要包括肾病学家、肾脏病理学家和基因实验室在内的多学科方法，以帮助改善这些患者的整体护理并制定最佳治疗方案。
