Boyce N W, Holdsworth S R
Nephron. 1986;44(1):22-5. doi: 10.1159/000183906.
A 58-year-old woman was hospitalized with acute renal failure and unilateral pulmonary consolidation. Ten days later she developed massive pulmonary haemorrhage with diffuse pulmonary consolidation. Renal biopsy revealed 100% crescentic nephritis without immunofluorescence (IF) or electron microscopic evidence of immune reactant deposition. Circulating anti-glomerular basement membrane (antiGBM) antibody was not detectable by radioimmunoassay. Despite aggressive therapy pulmonary haemorrhage eventually proved fatal. IF of lung tissue revealed no immune-reactant deposition. This report represents a case of idiopathic Goodpasture's syndrome, both from an aetiological and an immunopathological viewpoint. It emphasizes that Goodpasture's syndrome (i.e. pulmonary haemorrhage and glomerulonephritis) occurs in a variety of situations which are not mediated by antiGBM antibody deposition and that alveolar haemorrhage should be considered in the differential diagnosis of all radiological pulmonary infiltrates, including unilateral opacities, when abnormalities of renal function coexist.
一名58岁女性因急性肾衰竭和单侧肺实变入院。10天后，她出现大量肺出血并伴有弥漫性肺实变。肾活检显示100%为新月体性肾炎，免疫荧光（IF）或电子显微镜检查均未发现免疫反应物沉积的证据。放射免疫分析未检测到循环抗肾小球基底膜（antiGBM）抗体。尽管进行了积极治疗，肺出血最终还是导致了死亡。肺组织的IF检查未发现免疫反应物沉积。从病因学和免疫病理学角度来看，本报告代表了一例特发性古德帕斯彻综合征。它强调，古德帕斯彻综合征（即肺出血和肾小球肾炎）可发生在多种并非由antiGBM抗体沉积介导的情况下，并且当存在肾功能异常时，在所有影像学肺部浸润（包括单侧混浊）的鉴别诊断中都应考虑肺泡出血。
