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古德帕斯彻综合征:两例早期病程与治疗情况对比的病例

Goodpasture's syndrome: two cases with contrasting early course and management.

作者信息

Teichman S, Briggs W A, Knieser M R, Enquist R W

出版信息

Am Rev Respir Dis. 1976 Feb;113(2):223-32. doi: 10.1164/arrd.1976.113.2.223.

Abstract

In 2 patients with hemoptysis and hematuria, Goodpasture's syndrome was documented by clinical presentation, demonstration of circulating anti-glomerular basement membrane antibodies, and demonstration of linear immunofluorescence along glomerular and alveolar basement membranes. One patient was considered to have progressive disease and was treated with prednisone and cyclophosphamide. During the subsequent 18-month follow-up period, clinical remission occurred with eventual disappearance of hematuria and detectable circulating anti-glomerular basement membrane antibodies. The other patient did not have active hemoptysis at the time of referral and no therapy was instituted. After 12 months of follow-up, hemoptysis remains quiescent but hematuria and positive circulating anti-glomerular basement membrane antibodies persist. Although therapy appears to have been particularly effective in one patient, the benign course of the other patient precludes premature enthusiasm for early intervention.

摘要

在2例咯血和血尿患者中,通过临床表现、循环抗肾小球基底膜抗体的检测以及沿肾小球和肺泡基底膜的线性免疫荧光检测,确诊为Goodpasture综合征。1例患者被认为患有进行性疾病,接受了泼尼松和环磷酰胺治疗。在随后的18个月随访期内,临床症状缓解,血尿最终消失,循环抗肾小球基底膜抗体检测不到。另1例患者转诊时无活动性咯血,未进行治疗。随访12个月后,咯血仍处于静止状态,但血尿和循环抗肾小球基底膜抗体阳性持续存在。尽管治疗在1例患者中似乎特别有效,但另1例患者的良性病程不支持过早热衷于早期干预。

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