FOCAL SEGMENTAL GLOMERULAR SCLEROSIS IN ADULTS - PRESENTATION, COURSE, AND RESPONSE TO TREATMENT
FOCAL SEGMENTAL GLOMERULAR SCLEROSIS IN ADULTS - PRESENTATION, COURSE, AND RESPONSE TO TREATMENT
复制标题
DOI：
10.1016/0272-6386(95)90120-5
复制
发表时间：
1995-04-01
影响因子：
13.2
通讯作者：
SCHWARTZ, MM
中科院分区：
文献类型：
--
作者：
RYDEL, JJ;KORBET, SM;SCHWARTZ, MM
作者对81例原发性局灶节段性肾小球硬化（FSGS）患者进行了一项回顾性临床病理学研究，以确定他们是否可以在临床表现时识别出可预测结局和治疗反应的临床或组织学特征。男性占患者的58%，53%是黑人。活检时，患者年龄为40 +/- 17岁; 74%为肾病，62%存在肾功能不全。从发病到活检的平均时间为16个月，平均总随访时间为62个月。与非肾病患者相比，肾病患者的预后明显较差（5年和10年生存率分别为76%和57% vs92%和92%; P < 0.05）。对活检时的组织学和临床特征进行多变量分析，评估导致终末期肾病的危险因素，仅显示血清肌酐和间质纤维化程度具有显著相关性。30例肾病患者接受泼尼松治疗，治疗时间为5.5 ± 4个月，每个疗程的总剂量为5.9 ± 2.9 g。其中15例患者（50%）在3.7 +/- 2个月内达到缓解（10例完全缓解和5例部分缓解），所有患者均在9个月内缓解。只有两名患者自发缓解（均为部分缓解）。缓解期患者5年和10年生存率均为100%，未缓解期患者5年和10年生存率分别为66%和41%（P < 0.01）。在进行多变量分析时，没有临床表现或活检的临床特征可以预测对治疗的反应。对治疗有反应的肾病患者比无反应的患者有明显更好的预后。对治疗的反应需要至少3至4个月的泼尼松治疗。不幸的是，没有临床特征或活检预测哪些患者更可能对治疗有反应。这一经验表明，原发性FSGS的肾病患者可能受益于更长的泼尼松治疗疗程。此外，这些发现强调了需要在FSGS肾病患者中进行对照试验，以确认对治疗的反应并建立治疗指南。(C)1995年由国家肾脏基金会，公司。
The authors performed a retrospective clinicopathologic study in 81 patients with primary focal segmental glomerular sclerosis (FSGS) to determine whether they could identify clinical or histologic features at presentation that could be predictive of outcome and response to therapy. Males constituted 58% of patients, and 53% were black. At biopsy the patients were 40 +/- 17 years old; 74% were nephrotic, and renal insufficiency was present in 62%. The average time from presentation to biopsy was 16 months, and the average total follow-up was 62 months. Nephrotic patients had a significantly poorer prognosis as compared with nonnephrotic patients (5- and 10-year survivals of 76% and 57% v 92% and 92%; P < 0.05). A multivariate analysis was done on histologic and clinical features at biopsy, assessing for risk factors leading to end-stage renal disease, showing only the serum creatinine and the degree of interstitial fibrosis to have a significant correlation. Thirty nephrotic patients received prednisone, with a treatment time of 5.5 +/- 4 months and a total dose of 5.9 +/- 2.9 g per course of treatment. Fifteen of these patients (50%) achieved a remission by 3.7 +/- 2 months (10 complete remission and 5 partial remissions), with all patients responding within 9 months. Only two patients had spontaneous remissions (both partial). The 5- and 10-year survival for patients in remission were both 100% as compared with 66% and 41% (P < 0.01), respectively, for nephrotic patients not in remission. No clinical feature at presentation or biopsy was predictive of response to therapy when a multivariate analysis was performed. Nephrotic patients responding to therapy have a significantly better prognosis than nonresponsive patients. Response to therapy requires at least 3 to 4 months of treatment with prednisone. Unfortunately, no clinical feature at presentation or biopsy predicts which patients are more likely to respond to treatment. This experience suggests that nephrotic patients with primary FSGS may benefit from a more prolonged course of therapy with prednisone. Furthermore, these findings underscore the need for a controlled trial in nephrotic patients with FSGS to confirm the response to and establish guidelines for therapy. (C) 1995 by the National Kidney Foundation, Inc.