Koopman J J E, Teng Y K O, Boon C J F, van den Heuvel L P, Rabelink T J, van Kooten C, de Vries A P J
Section of Nephrology, Department of Internal Medicine, Leiden University Medical Center, Leiden, the Netherlands.
Neth J Med. 2019 Jan;77(1):10-18.
C3 glomerulopathy is a rare renal disease that has been distinguished as a renal disease for about 10 years. It is caused by an excessive activation of the alternative complement pathway in the circulation, which leads to deposition of complement factor C3 in glomeruli. It is diagnosed based on clinical presentation, histological patterns in a kidney biopsy and tests of the complement pathways. It can closely resemble immune complexmediated glomerulonephritis and postinfectious glomerulonephritis. Renal failure develops in up to half of all patients within 10 years after presentation. A curative treatment is not available. Treatment relies on renoprotective measures, occasional immunosuppressive medication and experimental novel complement inhibitors. Because the disease is rare, its care and cure are concentrated in centers of expertise. Here we provide an overview of the state-ofthe-art diagnosis and treatment of C3 glomerulopathy in a center of expertise in the Netherlands.
C3肾小球病是一种罕见的肾脏疾病，作为一种肾脏疾病已被区分出来约10年。它是由循环中替代补体途径的过度激活引起的，这导致补体因子C3在肾小球中沉积。它是根据临床表现、肾活检的组织学模式和补体途径检测来诊断的。它可能与免疫复合物介导的肾小球肾炎和感染后肾小球肾炎极为相似。高达半数的患者在出现症状后的10年内会发展为肾衰竭。目前尚无治愈性治疗方法。治疗依赖于肾脏保护措施、偶尔使用的免疫抑制药物以及实验性新型补体抑制剂。由于该疾病罕见，其护理和治疗集中在专业中心。在此，我们概述了荷兰一个专业中心对C3肾小球病的最新诊断和治疗情况。
