Berti F, Carnabuci A, Onetti A, Polchi S, Sorrentino R
Divisione di Medicina Interna Cesalpino I, Azienda Ospedaliera Nicholas Green, Roma.
Recenti Prog Med. 1997 Jan;88(1):26-31.
Goodpasture's disease is characterized by lung haemorrhage, associated with glomerulus basement membrane antibody glomerulonephritis, and circulating basement membrane antibody. Other diseases (Wegener, LES, arteritis) may have the same kidney and lung involvement. The Authors present a clinical case of rapidly progressive renal failure where renal biopsy showed an extensive extracapillary proliferative glomerulonephritis with linear deposits of antibody in the basement membrane, similar to Goodpasture's disease, with following lung involvement, but without hemoptysis and in absence of circulating antiglomerular basement membrane antibody. The Authors think it could be a case of Goodpasture's disease, even if it did not show the above-mentioned symptoms, whether out of the characteristic clinical course or the exclusion of all the other diseases. The Authors believe that the absence of circulating basement membrane antibody could be due to their sediment in the target organs and suggest a revision of the standards required for the Goodpasture's disease diagnosis.
肺出血肾炎综合征的特征是肺出血，伴有肾小球基底膜抗体性肾小球肾炎和循环性基底膜抗体。其他疾病（韦格纳肉芽肿、狼疮、动脉炎）可能有相同的肾脏和肺部受累情况。作者报告了一例快速进展性肾衰竭的临床病例，肾活检显示广泛的毛细血管外增生性肾小球肾炎，基底膜有抗体线性沉积，类似于肺出血肾炎综合征，随后出现肺部受累，但无咯血且无循环抗肾小球基底膜抗体。作者认为这可能是一例肺出血肾炎综合征病例，即使未表现出上述症状，无论是出于特征性临床病程还是排除了所有其他疾病。作者认为循环性基底膜抗体的缺失可能是由于它们沉积在靶器官中，并建议修订肺出血肾炎综合征诊断所需的标准。
