Schachter M E, Monahan M, Radhakrishnan J, Crew J, Pollak M, Ratner L, Valeri A M, Stokes M B, Appel G B
Division of Nephrology, Dept. of Medicine, St. Paul's Hospital, Vancouver, Canada.
Clin Nephrol. 2010 Sep;74(3):173-81. doi: 10.5414/cnp74173.
FSGS is an important cause of ESRD and tends to recur in allografts (rFSGS). Older series suggest recurrence rates of 30-60%. In the modern era of transplant immunosuppression, recurrence rates are unknown. There are also few data regarding prevalence of known genetic mutations in adult FSGS patients who undergo transplantation. Recently, FSGS has been subdivided into histological variants, which may predict renal outcomes; there is little information on patterns of recurrence and outcomes in these variants. Finally, treatment for rFSGS relies upon up-titrating calcineurin inhibitors and plasmapheresis. Insufficient information exists on the use of these regimens for rFSGS in the era of modern immunosuppression. We conducted a retrospective chart review involving all renal transplant recipients at Columbia University Medical Center from December 1999 to March 2007. Those with biopsy confirmed primary FSGS were included and information regarding baseline characteristics, histologic variants, genetics, treatment, and clinical outcomes were collected. FSGS recurred in 23% of patients. Those with collapsing histology on native kidney biopsy, tended to recur with the same histology. No known genetic mutations were identified among those with recurrence. Plasmapheresis resulted in complete or partial remission in 75% of those with recurrence. Recurrent FSGS resulted in a trend toward the combined outcome of ESRD or death compared to those without recurrence (27% vs. 12%). Modern immunosuppression does not reduce the rate of rFSGS, known genetic mutations are uncommon in such adult patients, collapsing FSGS tends to recur with the same histology, and plasmapheresis may be helpful in the treatment of recurrence.
局灶节段性肾小球硬化(FSGS)是终末期肾病(ESRD)的重要病因,且易在同种异体移植肾中复发(复发性FSGS,rFSGS)。较早的系列研究表明复发率为30%-60%。在现代移植免疫抑制时代,复发率尚不清楚。关于接受移植的成年FSGS患者中已知基因突变的患病率的数据也很少。最近,FSGS已被细分为组织学亚型,这可能预测肾脏预后;关于这些亚型的复发模式和预后的信息很少。最后,rFSGS的治疗依赖于增加钙调神经磷酸酶抑制剂的剂量和进行血浆置换。在现代免疫抑制时代,关于这些治疗方案用于rFSGS的信息不足。我们对1999年12月至2007年3月在哥伦比亚大学医学中心的所有肾移植受者进行了一项回顾性病历审查。纳入经活检证实为原发性FSGS的患者,并收集有关基线特征、组织学亚型、遗传学、治疗和临床结局的信息。23%的患者出现FSGS复发。那些在原肾活检中表现为塌陷性组织学的患者,倾向于以相同的组织学复发。复发患者中未发现已知的基因突变。血浆置换使75%的复发患者完全或部分缓解。与未复发的患者相比,复发性FSGS导致ESRD或死亡的综合结局有增加趋势(27%对12%)。现代免疫抑制并不能降低rFSGS的发生率,已知基因突变在这类成年患者中并不常见,塌陷性FSGS倾向于以相同的组织学复发,血浆置换可能有助于治疗复发。