抗肾小球基底膜病临床病理及血浆置换疗效分析

目的 分析抗肾小球基底膜（GBM）病的临床病理特点和预后；评价双膜血浆置换（DFPP）清除抗GBM抗体的有效性和安全性。 方法 回顾分析北京协和医院1999年10月至2010年5月确诊为抗GBM病的35例住院患者的临床病理资料。患者根据临床表现分为3组：组Ⅰ：24例严重肺出血或急进型肾小球肾炎（RPGN）者，接受甲泼尼龙（7.5~15 mg·kg-1·d-1，3~5 d）冲击和（或）DFPP治疗，后续以泼尼松（1.0 mg·kg-1·d-1）和（或）环磷酰胺（CTX 0.1 g/d）；组Ⅱ：5例无严重肺出血或RPGN者予泼尼松和（或）CTX治疗；组Ⅲ：5例就诊时已为终末期肾病（ESRD）和1例肾功能正常者未给予免疫抑制治疗。观察患者临床病理特点，连续监测4例患者DFPP治疗前后抗GBM抗体滴度变化情况，计算抗体的清除率。分析影响预后的相关因素。 结果 35例患者平均年龄（41.06±16.55）岁，男女比例4∶3；16例（45.7%）患者表现为Goodpasture综合征；18例（51.4%）表现为抗GBM肾小球肾炎。24例接受肾穿刺活检患者中，13例（54.2%）表现为新月体肾小球肾炎；7例患者并发其他肾小球肾炎。组Ⅰ死亡7例，50%患者肾脏长期存活。与组Ⅱ相比，组Ⅰ患者入院时Scr水平、抗GBM抗体滴度、肾小球新月体比例均显著升高（P < 0.05）；老年患者、贫血、入院时Scr水平高（>300 μmol/L)及硬化肾小球比例更高；入院时少尿或无尿、需要血液透析治疗、肾脏预后差更普遍。18例患者的94次DFPP治疗中，无明显出血、低血压；4例连续动态监测抗GBM抗体滴度的患者中，4~6次DFPP后抗GBM抗体转阴，中位清除率为55%。 结论 根据不同临床表现选择个体化的治疗方案有助于改善预后，减少并发症。DFPP能安全有效地清除抗GBM抗体。

Analysis of clinicopathology and plasmapheresis efficacy in patients with anti-glomerular basement membrane disease

objective To analyze the clinicopathological features and prognosis of antiglomerular basement membrane(GBM)disease,and evaluate the efficacy and safety of double filtration plasmapheresis(DFPP). Methods A total of 35 hospitalized patients diagnosed as anti-GBM disease in our department were enrolled in the study.All the patients were divided into 3 groups according to the manifestations at admission.Group Ⅰ∶24 patients with severe pulmonary hemorrhage or rapidly progressive glomerulonephritis(RPGN)received pulse methylprednisolone with or without DFPP,and then followed by prednisone and CTX.Group Ⅱ∶5 patients without severe pulmonary hemorrhage and RPGN received prednisone and CTX.Group Ⅲ∶5 ESRD patients and 1 normal renal function patient did not receive immunosuppression therapy.Anti-GBM antibody titer of pre-and post-DFPP in 4 patients was measured consecutively,and removal rate was calculated.Results The mean age of all the patients was(41.1±16.6)years.Sixteen patients(45.7%)presented Goodpasture's syndrome.Eighteen patients(51.4%)had anti-GBM glomerulonephritis alone,whereas one suffered solely from pulmonary hemorrhage.20%patients had positive P-ANCA serology.54.2%crescentic glomerulonephritis and 7 with other glomerulonephritis were revealed by kidney biopsy in 24 patients.Patients in Group Ⅰ showed more severe manifestation at admission:higher Scr level,higher titer of anit-GBM antibody,greater percentage of crescents.Within the follow-up period,7 patients died and kidneys of 50%patients survived.No patient died in Group Ⅱ and Ⅲ.The elder age,anemia,higher Scr(＞300 μmol/L),oliguria or anuria,emergency hemodialysis at admission,and more glomerular sclerosis were predictors of poor prognosis.The anti-GBM antibody was negative after 4 to 6 sessions of DFPP.and the mean removal rate was 55%.During total 94 DFPP sessions,there was no unacceptable morbidity. Conclusions Different therapy strategy is necessary for anti-GBM disease with different clinical manifestations.DFPP is an effective and safe clearance way of anti-GBM antibody.