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新月体性抗肾小球基底膜抗体(抗GBM)肾炎/肺出血肾炎综合征治疗的临床与形态学方面

Clinical and morphological aspects of the management of crescentic anti-glomerular basement membrane antibody (anti-GBM) nephritis/Goodpasture's syndrome.

作者信息

Walker R G, Scheinkestel C, Becker G J, Owen J E, Dowling J P, Kincaid-Smith P

出版信息

Q J Med. 1985 Jan;54(213):75-89.

PMID:3975346
Abstract

Twenty-two patients with crescentic anti-GBM nephritis or Goodpasture's syndrome with renal impairment were reviewed. All patients were treated with a combination of plasma exchange and immunosuppression. Sixteen patients (73 per cent) showed improvement in renal function (greater than 30 per cent reduction in serum creatinine level) apparently in response to treatment, and nine patients (41 per cent) made long-term recoveries in renal function. The most important features carrying a bad prognosis were total anuria, and/or a very high percentage of glomeruli showing crescents (greater than 85 per cent) in the initial renal biopsy. Some patients with other so-called 'bad' prognostic features, including severely impaired renal function at presentation, oliguria and the need to institute dialysis had unexpected marked improvement in renal function and/or recovered renal function in the long term provided treatment with plasma exchange was begun promptly and maintained for a sufficient period to allow resolution of the disease process. Renal biopsies at the beginning and later proved to be an extremely valuable guide for the progress and outcome of the disease.

摘要

对22例患有新月体性抗肾小球基底膜肾炎或肺出血肾炎综合征且伴有肾功能损害的患者进行了回顾性研究。所有患者均接受了血浆置换和免疫抑制联合治疗。16例患者(73%)肾功能有所改善(血清肌酐水平降低超过30%),显然是对治疗有反应,9例患者(41%)肾功能实现了长期恢复。预后不良的最重要特征是初始肾活检时出现完全无尿和/或极高比例的肾小球呈现新月体(超过85%)。一些具有其他所谓“不良”预后特征的患者,包括就诊时肾功能严重受损、少尿以及需要进行透析,只要及时开始血浆置换治疗并维持足够长的时间以使疾病进程得到缓解,其肾功能会有意外的显著改善和/或长期恢复。最初及之后的肾活检结果被证明是疾病进展和预后的极有价值的指导。

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