Moreso F, Poveda R, Gil-Vernet S, Carreras L, García-Osuna R, Griñó J M, Alsina J
Servicio de Nefrología, Ciudad Sanitaria y Universitaria de Vellvitge, L'Hospitalet de Llobregat, Barcelona.
Med Clin (Barc). 1995 Jun 10;105(2):59-61.
The extraction of circulating antiglomerular basement membrane (GBM) antibodies by plasmapheresis (PP) has allowed the prognosis of Goodpasture's disease to markedly improve. Immunoabsorption (IA) may improve the results of PP upon allowing more effective immunoglobulin extraction. Two patients with Goodpasture disease were treated with IA. A rapid decrease was observed in the serum levels of anti GBM antibodies and improvement in respiratory failure. In one of the patients this regimen was administered following the observation of a lack of clinical response of pulmonary hemorrhage in three PP sessions (9 liters of treated plasma). In this patient, the IA processed 39 liters of plasma and the method was found to be equally effective on reinstatement (29 liters) on the occasion of a relapse in the pulmonary symptoms presented at three weeks after the first treatment. Both cases showed renal involvement. In one case this was incipient and the treatment was associated with non progression of the kidney disease, normalization of the urine sediment and preservation of renal function. In the second case treatment was initiated at an advanced disease state with no changes in dialysis needs. Immunoadsorption has shown to be effective in the treatment of pulmonary hemorrhage in Goodpasture's disease. Onset of treatment at an early stage of the kidney disease may avoid progression to renal failure.
通过血浆置换(PP)提取循环抗肾小球基底膜(GBM)抗体已使Goodpasture病的预后得到显著改善。免疫吸附(IA)在更有效地提取免疫球蛋白时可能会改善血浆置换的效果。两名Goodpasture病患者接受了免疫吸附治疗。观察到抗GBM抗体血清水平迅速下降,呼吸衰竭得到改善。其中一名患者在进行了三次血浆置换(9升处理过的血浆)后,肺部出血缺乏临床反应,随后采用了该治疗方案。在该患者中,免疫吸附处理了39升血浆,并且在首次治疗三周后出现肺部症状复发时,发现该方法在恢复治疗(29升)时同样有效。两例均有肾脏受累。在一例中为早期,治疗与肾病无进展、尿沉渣正常化及肾功能保留有关。在第二例中,治疗在疾病晚期开始,透析需求无变化。免疫吸附已显示对Goodpasture病的肺部出血治疗有效。在肾病早期开始治疗可能避免进展至肾衰竭。