Simpson I J, Doak P B, Williams L C, Blacklock H A, Hill R S, Teague C A, Herdson P B, Wilson C B
Am J Nephrol. 1982;2(6):301-11. doi: 10.1159/000166666.
The clinical course and levels of anti-glomerular basement membrane (GBM) antibody were compared in 20 patients with Goodpasture's syndrome treated with plasma exchange and immunosuppression (8 patients), immunosuppression alone (4 patients) or no specific therapy (8 patients). There was a more rapid fall in the level of anti-GBM antibody and pulmonary hemorrhage was less protracted in the 8 patients treated with plasma exchange and immunosuppression. In this group, 1 patient who presented with severe renal failure showed a marked improvement of renal function and there was no progression of disease in the 4 with milder renal involvement. 2 of the 4 patients treated with immunosuppression alone, and only 2 of the 8 patients who received no specific therapy, maintained normal renal function. In the group which received no specific therapy, 1 of the 6 patients who progressed to renal failure had mild renal involvement initially. There was a significant correlation between the level of anti-GBM antibody and the severity of the morphological changes seen at renal biopsy but not between the level of anti-GBM antibody and the severity of lung hemorrhage. The course and outcome of the disease in those patients not treated, or treated with immunosuppression alone, was better than that described in early reports of this disease, while those patients with plasma exchange and immunosuppression fared even better. An adequately stratified controlled trial of immunosuppression and plasma exchange versus immunosuppression alone is in order.
对20例Goodpasture综合征患者进行了比较，这些患者分别接受了血浆置换联合免疫抑制治疗（8例）、单纯免疫抑制治疗（4例）或未接受特异性治疗（8例），比较了他们的临床病程及抗肾小球基底膜（GBM）抗体水平。接受血浆置换联合免疫抑制治疗的8例患者，其抗GBM抗体水平下降更快，肺出血持续时间更短。在该组中，1例出现严重肾衰竭的患者肾功能显著改善，4例肾受累较轻的患者病情未进展。单纯接受免疫抑制治疗的4例患者中，2例维持了正常肾功能；未接受特异性治疗的8例患者中，也只有2例维持了正常肾功能。在未接受特异性治疗的组中，6例进展为肾衰竭的患者中有1例最初肾受累较轻。抗GBM抗体水平与肾活检所见形态学改变的严重程度之间存在显著相关性，但与肺出血的严重程度无关。未接受治疗或仅接受免疫抑制治疗的患者的病程和结局比该疾病早期报告中描述的要好，而接受血浆置换联合免疫抑制治疗的患者情况更好。有必要进行一项关于免疫抑制和血浆置换与单纯免疫抑制的充分分层对照试验。
