Holdsworth S, Boyce N, Thomson N M, Atkins R C
Q J Med. 1985 Apr;55(216):75-86.
The aetiology, clinical features and outcome of 40 patients presenting with Goodpasture's syndrome (glomerulonephritis with haemoptysis and pulmonary infiltrates) are reviewed. The diseases of the patients studied could be divided into three groups: antiglomerular basement membrane (anti-GBM) antibody-induced disease (7/40); systemic vasculitis (22/40) and idiopathic Goodpasture's syndrome (i.e. no systemic disease or anti-GBM antibody detected) (11/40). Overall mortality was 57.5 per cent (anti-GBM disease 4/7; systemic vasculitis 15/22; and idiopathic Goodpasture's syndrome 4/11). Most patients died of disease progression or infection. End-stage renal failure developed in 26 patients (anti-GBM (7), vasculitis (14) and idiopathic Goodpasture's syndrome (5). End-stage renal failure developed in 23 of 24 patients presenting with a creatinine of greater than 600 microM/l regardless of the aetiology of Goodpasture's syndrome or treatment used. Review of renal histology showed that all had proliferative nephritis, with 80 per cent of patients having more than 30 per cent crescents. Thus Goodpasture's syndrome was associated with a wide variety of underlying disease. It had a poor prognosis, with the degree of renal impairment at presentation, the extent of crescent formation and the nature of the underlying disease being the major determinants of outcome.
对40例患有古德帕斯彻综合征（肾小球肾炎伴咯血和肺部浸润）患者的病因、临床特征及预后进行了回顾。所研究患者的疾病可分为三组：抗肾小球基底膜（anti-GBM）抗体诱导的疾病（7/40）；系统性血管炎（22/40）和特发性古德帕斯彻综合征（即未检测到系统性疾病或抗GBM抗体）（11/40）。总死亡率为57.5%（抗GBM疾病4/7；系统性血管炎15/22；特发性古德帕斯彻综合征4/11）。大多数患者死于疾病进展或感染。26例患者出现终末期肾衰竭（抗GBM（7例）、血管炎（14例）和特发性古德帕斯彻综合征（5例））。无论古德帕斯彻综合征的病因或所采用的治疗方法如何，24例血肌酐大于600微摩尔/升的患者中有23例出现终末期肾衰竭。对肾脏组织学的回顾显示，所有患者均有增殖性肾炎，80%的患者新月体超过30%。因此古德帕斯彻综合征与多种潜在疾病相关。其预后较差，就诊时的肾功能损害程度、新月体形成的程度以及潜在疾病的性质是预后的主要决定因素。
