Briggs W A, Johnson J P, Teichman S, Yeager H C, Wilson C B
Medicine (Baltimore). 1979 Sep;58(5):348-61. doi: 10.1097/00005792-197909000-00002.
The originally dismal prognosis associated with anti-GBM Ab-mediated GN and Goodpasture's syndrome may be changing as we recognize a broader spectrum of disease, improve general supportive care, and improve specific treatment. Immunosuppressive therapy, if started early in the course of disease, may prevent or allow recovery from renal failure and may also result in cessation of recurrent pulmonary hemorrhage in most patients with this form of Goodpasture's syndrome. The administration of pharmacologic doses of corticosteroids intravenously can result in cessation of and dramatic recovery from severe pulmonary hemorrhage and obviate the need for emergency bilateral nephrectomy. Plasmaspheresis may represent a useful therapeutic procedure for the immediate and long term reduction in amounts of circulating anti-GBM Ab, but the definition of its true value and role awaits completion of controlled, prospective trials. Immunosuppressive therapy, with or without plasmapheresis, can reduce quantities of anti-GBM Ab in serum to undetectable levels without nephrectomy. Thus, it is likely, but not proven, that nephrectomy can be discontinued as a routine pretransplantation procedure in patients with anti-GBM Ab mediated GN. Finally, in patients who suffer irreversible renal failure, renal transplantation can be successfully undertaken with minimal risk of recurrent disease, when circulating anti-GBM Ab becomes undetectable.
随着我们对疾病谱认识的拓宽、一般支持治疗的改善以及特异性治疗的进步,抗肾小球基底膜抗体(anti-GBM Ab)介导的肾小球肾炎(GN)和肺出血肾炎综合征原本预后不佳的情况可能正在发生改变。免疫抑制治疗若在疾病早期开始,可能预防肾衰竭或使其恢复,也可能使大多数此类肺出血肾炎综合征患者反复发生的肺出血停止。静脉给予药理剂量的糖皮质激素可使严重肺出血停止并显著恢复,从而无需进行急诊双侧肾切除术。血浆置换可能是一种有用的治疗方法,可立即并长期减少循环中的anti-GBM Ab量,但其真正价值和作用的界定有待对照前瞻性试验的完成。免疫抑制治疗,无论是否联合血浆置换,均可在不进行肾切除术的情况下将血清中的anti-GBM Ab量降至检测不到的水平。因此,抗GBM Ab介导的GN患者作为常规移植前操作的肾切除术有可能(但尚未得到证实)可以停用。最后,对于发生不可逆肾衰竭的患者,当循环中的anti-GBM Ab检测不到时,肾移植可成功进行,且疾病复发风险极小。