Lockwood C M, Rees A J, Pearson T A, Evans D J, Peters D K, Wilson C B
Lancet. 1976 Apr 3;1(7962):711-5. doi: 10.1016/s0140-6736(76)93089-0.
Seven patients with Goodpasture's syndrome induced by anti-glomerular-basement-membrane (anti-G.B.M.) antibody were treated by a regimen of intensive plasma-exchange, cytotoxic drugs, and steroids. In the three patients retaining some renal function at presentation, this regimen led to suppression and eventual termination of antibody synthesis with improvement in renal function. In four patients, all anuric at presentation, antibody to G.B.M. persisted with variable reduction in the circulating levels. No return of renal function occurred in this group, all of whom had extensive changes on renal biopsy. Pulmonary haemorrhage, life-threatening in one patient, was rapidly controlled in all five patients in whom it was a presenting feature. In addition to its effect on antibody levels, plasma-exchange, using volume-replacement with plasma-protein fraction (P.P.F.), resulted in substantial depletion of complement and fibrinogen, mediators possibly contributing to the antibody-induced injury.
7例由抗肾小球基底膜(anti-GBM)抗体诱发的Goodpasture综合征患者接受了强化血浆置换、细胞毒性药物和类固醇治疗方案。在3例初诊时仍保留部分肾功能的患者中,该方案导致抗体合成受到抑制并最终终止,肾功能得到改善。在4例初诊时均无尿的患者中,抗GBM抗体持续存在,循环水平有不同程度降低。该组患者均未恢复肾功能,所有患者肾活检均有广泛病变。肺出血在1例患者中危及生命,在所有以肺出血为首发症状的5例患者中均迅速得到控制。除了对抗体水平有影响外,使用血浆蛋白组分(PPF)进行容量置换的血浆置换导致补体和纤维蛋白原大量消耗,这些介质可能促成了抗体诱导的损伤。