Treatment and outcome of adult patients with primary focal segmental glomerulosclerosis in five UK renal units
Treatment and outcome of adult patients with primary focal segmental glomerulosclerosis in five UK renal units
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DOI：
10.1093/qjmed/hci072
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发表时间：
2005-06-01
影响因子：
13.3
通讯作者：
Adu, D
中科院分区：
文献类型：
--
作者：
Stirling, CM;Mathieson, P;Adu, D
背景资料：局灶节段性肾小球硬化（FSGS）是原发性肾病综合征的病因中研究最少的一种，并且很少有具体的治疗指南。目的：回顾来自英国五个肾脏单位的数据，调查FSGS成人患者是否得到统一的治疗，并检查治疗对蛋白尿和生存率的影响。设计：回顾性记录回顾。方法：我们检查了特发性FSGS患者的电子记录，以了解基线临床参数、治疗方案和结局。在136例原发性FSGS和肾病范围蛋白尿患者中，76例（56%）接受了泼尼松龙治疗，其中59%接受了额外的免疫抑制治疗。在接受治疗的患者中，总缓解率（完全和部分）为67%，一家医院的缓解率达到80%。治疗患者的缓解率明显高于未治疗患者。缓解与94%的透析后5年生存率相关，而如果未达到缓解，则为53%。基线血清肌酐和缓解独立相关的生存透析在一个多变量考克斯比例风险model.Discussion：原发性FSGS和肾病范围蛋白尿，谁是皮质类固醇治疗的患者，更有可能进入缓解比那些谁不治疗。通过延长治疗可以达到高达80%的缓解率，缓解是透析后生存率的独立预测因素。未达到缓解的患者预后不良。进一步阐明最佳治疗方案需要额外的前瞻性研究。
Background: Focal segmental glomerulosclerosis (FSGS) is the least studied of the causes of idiopathic nephrotic syndrome, and there are few specific guidelines for treatment.Aim: To review data from five UK renal units to investigate whether adult patients with FSGS were treated uniformly, and to examine the effect of treatment on proteinuria and survival.Design: Retrospective record review.Methods: We examined electronic records of patients with idiopathic FSGS for information on baseline clinical parameters, treatment regimens and outcomes.Results: Of 136 patients with primary FSGS and nephrotic range proteinuria, 76 (56%) were treated with prednisolone and of this group, 59% were treated with additional immunosuppression. Among the treated patients, the total remission rate (complete and partial) was 67%, and one hospital achieved a remission rate of 80%. Treated patients had a significantly higher remission rate than those who were not treated. Remission was associated with a 5-year survival off dialysis of 94%, compared with 53% if remission was not achieved. Baseline serum creatinine and remission were independently associated with survival off dialysis in a multivariate Cox proportional hazards model.Discussion: Patients with primary FSGS and nephrotic range proteinuria, who are treated with corticosteroids, are more likely to enter remission than those who are not treated. Remission rates of up to 80% can be achieved with prolonged treatment, and remission is an independent predictor of survival off dialysis. Patients who do not achieve remission have a poor prognosis. Further clarification of optimal treatment regimens requires additional, prospective studies.