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风湿性疾病继发肾淀粉样变性的烷化剂细胞毒性治疗

Alkylating cytostatic treatment in renal amyloidosis secondary to rheumatic disease.

作者信息

Berglund K, Keller C, Thysell H

机构信息

Department of Rheumatology, University Hospital, Lund, Sweden.

出版信息

Ann Rheum Dis. 1987 Oct;46(10):757-62. doi: 10.1136/ard.46.10.757.

Abstract

Fourteen consecutive patients with chronic inflammatory rheumatic disease and reactive renal amyloidosis were treated with alkylating cytostatics in 22 separate periods varying in duration between six and 30 months. Chlorambucil alone was given in 14 treatment periods, cyclophosphamide alone in six, and both alternately in two. The dosage was adjusted to attain a major suppression of the rheumatic inflammation and a blood lymphocyte level below 1.0 X 10(9)/l. Renal function improved in 12 treatment periods, renal deterioration was arrested in three periods, and in another four periods the rate of functional decline slowed down. In the remaining three treatment periods, associated with further deterioration in renal function, treatment was inadequate owing to blood dyscrasia and failure to control hypertension. Glomerular filtration rate (GFR) was followed more closely in 10 treatment periods, in all of which the falling trend was arrested or reduced. The survival rate at five years was 93%. Three patients who dropped out of the treatment programme are so far the only ones not still alive. Nine are still being followed up after 6-17 years, and the other two remaining live patients have had renal transplants for five years.

摘要

14例患有慢性炎性风湿性疾病和反应性肾淀粉样变性的连续患者在22个不同时期接受了烷化剂类细胞抑制剂治疗,治疗期持续时间在6至30个月之间。14个治疗期单独给予苯丁酸氮芥,6个治疗期单独给予环磷酰胺,2个治疗期二者交替使用。调整剂量以实现对风湿性炎症的主要抑制以及使血淋巴细胞水平低于1.0×10⁹/L。12个治疗期肾功能得到改善,3个治疗期肾功能恶化停止,另外4个治疗期功能下降速率减缓。在其余3个与肾功能进一步恶化相关的治疗期,由于血液恶病质和未能控制高血压,治疗不充分。在10个治疗期更密切地监测了肾小球滤过率(GFR),在所有这些治疗期中,下降趋势均停止或减缓。五年生存率为93%。退出治疗方案的3例患者是目前仅有的已死亡患者。9例患者在6至17年后仍在接受随访,另外2例存活患者已接受肾移植5年。

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