Department of Pathology (Analytic Human Pathology), Nippon Medical School, Tokyo, Japan.
Clin Transplant. 2011 Jul;25 Suppl 23:6-14. doi: 10.1111/j.1399-0012.2011.01452.x.
Focal segmental glomerulosclerosis (FSGS) is a clinicopathologic syndrome of proteinuria, usually of nephrotic range, associated with focal and segmental sclerotic glomerular lesions. Therefore, FSGS is diagnosed by clinical features and histopathological examination of renal biopsy. The natural history of the condition varies, and although it may respond to treatment, FSGS is an important disease in the etiology of end-stage renal disease (ESRD). Furthermore, after kidney transplantation, approximately 30% of patients with FSGS develop recurrent FSGS. The risk factors for recurrence of FSGS include childhood onset and age <15 yr, rapid progression of the primary FSGS to ESRD, recurrence of FSGS in a previous allograft, diffuse mesangial hypercellularity in the native kidney, collapsing FSGS, and podocin gene mutation. In addition, after kidney transplantation, de novo FSGS also develops in approximately 10-20% of allografts, associated with a complication of hyperfiltration injury, chronic transplant glomerulopathy, and calcineurin inhibitor toxicity. FSGS is considered a podocyte disease, and the pathology is characterized by segmental FSGS lesion with glomerular epithelial hypercellularity. The pathological diagnosis of FSGS is based on the 2004 Columbia classification system. In the present minireview, we discuss the pathology of recurrence and de novo FSGS after kidney transplantation.
局灶节段性肾小球硬化症(FSGS)是一种以蛋白尿为特征的临床病理综合征,通常为肾病范围,伴有局灶和节段性硬化性肾小球病变。因此,FSGS 通过临床特征和肾活检的组织病理学检查来诊断。该疾病的自然病程各不相同,尽管它可能对治疗有反应,但 FSGS 是导致终末期肾病(ESRD)的重要病因之一。此外,在肾移植后,约 30%的 FSGS 患者会出现复发性 FSGS。FSGS 复发的危险因素包括儿童发病和<15 岁、原发性 FSGS 快速进展至 ESRD、先前同种异体移植中 FSGS 复发、原发性肾脏弥漫性系膜细胞增生、塌陷性 FSGS 和足细胞基因突变。此外,在肾移植后,约 10-20%的同种异体移植中也会出现新发 FSGS,与超滤损伤、慢性移植性肾小球病和钙调神经磷酸酶抑制剂毒性等并发症有关。FSGS 被认为是一种足细胞疾病,其病理特征为节段性 FSGS 病变伴肾小球上皮细胞增生。FSGS 的病理诊断基于 2004 年哥伦比亚分类系统。在本次小型综述中,我们讨论了肾移植后复发性和新发 FSGS 的病理学。