Shilov E M, Tareeva I E, Ivanov A A, Lavrova O N, Krasnova T N, Khudova I Iu, Miroshnichenko N G, Ratner M Ia, Biriukova L S
Ter Arkh. 2000;72(6):21-5.
To retrospectively analyze clinical course and results of immunodepressive therapy of patients with primary focal-segmental glomerulosclerosis (FSGS), to reveal prognostic factors of the disease progression and patients' sensitivity to immunosuppressive therapy.
Morphological diagnosis was specified, morphological indices of activity and sclerosis were estimated, renal survival was analysed, mono- and multivariate analysis of prognostic factors was made by the evidence obtained in the study of 135 biopsy specimens from CRF patients meeting the criteria of FSGS.
At the moment of the disease onset only age of the patients was related to FSGS: 5- and 10-year survival was 100% if the disease started under 16 years of age, if older--the survival was 80 and 65%, respectively. Nephrotic syndrome, hematuria, high creatinine, racemose alterations in the glomeruli worsened the disease prognosis. When cytostatics and corticosteroids were used in combination they produced better results and were associated with better prognosis than each of them in monotherapy. Patients with marked hematuria and low proteinuria were less sensitive to therapy than those with weak hematuria and high proteinemia. Patients with FSGS having high IA and SI required more aggressive therapy for response.
Renal biopsy with quantitation of IA and IS increases the prognosis accuracy and is important for choice of the treatment policy in patients with primary FSGS.
回顾性分析原发性局灶节段性肾小球硬化(FSGS)患者免疫抑制治疗的临床过程及结果,揭示疾病进展的预后因素以及患者对免疫抑制治疗的敏感性。
明确形态学诊断,评估活动和硬化的形态学指标,分析肾脏存活率,通过对135例符合FSGS标准的慢性肾衰竭患者活检标本研究获得的证据进行单因素和多因素预后因素分析。
疾病发作时,仅患者年龄与FSGS相关:如果疾病在16岁以下开始,5年和10年生存率为100%,如果年龄较大,生存率分别为80%和65%。肾病综合征、血尿、高肌酐、肾小球的分叶状改变会使疾病预后恶化。当细胞抑制剂和皮质类固醇联合使用时,它们产生的效果更好,并且与单一疗法相比预后更好。血尿明显且蛋白尿低的患者对治疗的敏感性低于血尿轻微且蛋白血症高的患者。IA和SI高的FSGS患者需要更积极的治疗才能产生反应。
对IA和IS进行定量的肾活检可提高预后准确性,对原发性FSGS患者治疗策略的选择很重要。