免疫吸附治疗抗肾小球基膜抗体疾病——附5例报告

Staphylococcal protein A immunoadsorption for anti-glomerular basement membrane diseases——report of five cases

目的 :探讨免疫吸附 (IA)治疗对抗肾小球基膜抗体 (抗 GBM)疾病患者血清抗 GBM水平的影响及其临床疗效。 方法 :5例抗 GBM疾病患者 ,4例伴有明显肺出血 ,1例仅有肾损害 ,临床均表现为急进性肾炎 ,SCr312～ 12 90 μmol/L。肾活检显示肾小球新月体比例 2 6 3%～ 10 0 %。血清抗 GBM抗体 4 0 6 %～ 2 0 3 2RU/ml。在激素治疗同时 ,应用葡萄球菌A蛋白吸附柱进行免疫吸附治疗 ,一疗程共 10次 ,再生血浆 30～ 6 0L。 结果 :吸附治疗 10次后 ,3例血清抗 GBM抗体转阴 ,随访 4个月时抗 GBM抗体仍阴性。 1例吸附治疗后血清抗 GBM抗体仍阳性 ,但较治疗前下降 6 2 8% ,进行第二个疗程吸附后 ,抗 GBM抗体转阴。 4例伴有肺出血患者治疗后肺出血迅速消失。 4例治疗前需血液透析的患者治疗后 1例摆脱透析 ,SCr由 5 0 5 μmol/L降至 15 7μmol/L ,另 3例肾活检病理显示10 0 %新月体形成者仍需维持性血液透析。 1例治疗前不需血液透析者肾功能保持稳定。 结论 :葡萄球菌A蛋白免疫吸附能有效降低血清抗 GBM抗体水平 ,迅速缓解肺出血 。

Objective:To investigate the clinical efficacy of protein A immunoadsorption treatment (IA) in patients with anti glomerular basement membrane disease (Anti GBM) disease. Methodology:Five patients with Anti GBM disease (4 complicated with diffuse pulmonary hemorrhage) with rapidly progressive glomerulonephritis (serum creatinine levels 312～ 1 290 μmol/L) and pathologically diffusive crescentic nephritis ( crescent percentage 26.3%～100%) were included in this study. All patients had positive serum IgG Anti GBM antibodies in the range of 40.6～203.2 RU/ml by ELISA method. All patients received intravenous methyprednisolone pulse therapy and 10 cycles of immunoadsorption with Protein A column, with 3 000 ml plasma processed in each treatment cycle. Results:The levels of serum Anti GBM antibody decreased to normal in four patients after IA for 10 treatment cycles and remained negative during the first four months' follow up. While in the other 1 patient, serum Anti GBM did not turned negative although there was significant decrement of 62.8% at the end of 10 cycles of IA treatment (serum Anti GBM turned negative after another 10 cycles of IA treatment). Pulmonary hemorrhage resolved completely in all the 4 patients, including the one who was unresponsive to methylprednisolone pulse therapy before IA. Renal function was significantly improved in 1 of 4 patients who required haemodialysis prior to IA treatment, 3 patients whose renal biopsy showed 100% of glomerular crescents formation needed maintenance haemodialysis. Renal function remained stable in the patient whose serum creatinine levels not above that requiring haemodialysis before treatment. Conclusion:Immunoadsorption could rapidly decrease serum anti GBM antibody, resolve pulmonary hemorrhage and improve renal function.