中华肾脏病杂志
中華腎髒病雜誌
중화신장병잡지
2011年
4期
230-235
,共6页
牟利军%陈丽萌%左来孟%文煜冰%李航%秦岩%李明喜%陶建瓴%叶文玲%徐红%叶葳%孙阳%李雪梅%李学旺
牟利軍%陳麗萌%左來孟%文煜冰%李航%秦巖%李明喜%陶建瓴%葉文玲%徐紅%葉葳%孫暘%李雪梅%李學旺
모리군%진려맹%좌래맹%문욱빙%리항%진암%리명희%도건령%협문령%서홍%협위%손양%리설매%리학왕
肾小球基底膜%血浆置换%抗肾小球基底膜抗体%治疗%预后
腎小毬基底膜%血漿置換%抗腎小毬基底膜抗體%治療%預後
신소구기저막%혈장치환%항신소구기저막항체%치료%예후
Glomerular basement membrane%Plasma exchange%Anti-glomerular basement membrane antibody%Therapy%Prognosis
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抗体的有效件和安全性.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(＞300μmol/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.
目的 分析抗腎小毬基底膜(GBM)病的臨床病理特點和預後;評價雙膜血漿置換(DFPP)清除抗GBM抗體的有效件和安全性.方法 迴顧分析北京協和醫院1999年10月至2010年5月確診為抗GBM病的35例住院患者的臨床病理資料.患者根據臨床錶現分為3組:組Ⅰ∶24例嚴重肺齣血或急進型腎小毬腎炎(RPGN)者,接受甲潑尼龍(7.5～15 mg·kg-1·d-1,3～5 d)遲擊和(或)DFPP治療,後續以潑尼鬆(1.0 mg·kg-1·d-1)和(或)環燐酰胺(CTX 0.1 g/d);組Ⅱ∶5例無嚴重肺齣血或RPGN者予潑尼鬆和(或)CTX治療;組Ⅲ:5例就診時已為終末期腎病(ESRD)和1例腎功能正常者未給予免疫抑製治療.觀察患者臨床病理特點,連續鑑測4例患者DFPP治療前後抗GBM抗體滴度變化情況,計算抗體的清除率.分析影響預後的相關因素.結果 35例患者平均年齡(41.06±16.55)歲,男女比例4∶3∶16例(45.7%)患者錶現為Goodpasture綜閤徵;18例(51.4%)錶現為抗GBM腎小毬腎炎.24例接受腎穿刺活檢患者中,13例(54.2%)錶現為新月體腎小毬腎炎;7例患者併髮其他腎小毬腎炎.組Ⅰ,死亡7例,50%患者腎髒長期存活.與組Ⅱ相比,組Ⅰ患者入院時Scr水平、抗GBM抗體滴度、腎小毬新月體比例均顯著升高(P＜0.05);老年患者、貧血、入院時Scr水平高(＞300μmol/L)及硬化腎小毬比例更高;入院時少尿或無尿、需要血液透析治療、腎髒預後差更普遍.18例患者的94次DFPP治療中,無明顯齣血、低血壓;4例連續動態鑑測抗GBM抗體滴度的患者中,4～6次DFPP後抗GBM抗體轉陰,中位清除率為55%.結論 根據不同臨床錶現選擇箇體化的治療方案有助于改善預後,減少併髮癥.DFPP能安全有效地清除抗GBM抗體.
목적 분석항신소구기저막(GBM)병적림상병리특점화예후;평개쌍막혈장치환(DFPP)청제항GBM항체적유효건화안전성.방법 회고분석북경협화의원1999년10월지2010년5월학진위항GBM병적35례주원환자적림상병리자료.환자근거림상표현분위3조:조Ⅰ∶24례엄중폐출혈혹급진형신소구신염(RPGN)자,접수갑발니룡(7.5～15 mg·kg-1·d-1,3～5 d)충격화(혹)DFPP치료,후속이발니송(1.0 mg·kg-1·d-1)화(혹)배린선알(CTX 0.1 g/d);조Ⅱ∶5례무엄중폐출혈혹RPGN자여발니송화(혹)CTX치료;조Ⅲ:5례취진시이위종말기신병(ESRD)화1례신공능정상자미급여면역억제치료.관찰환자림상병리특점,련속감측4례환자DFPP치료전후항GBM항체적도변화정황,계산항체적청제솔.분석영향예후적상관인소.결과 35례환자평균년령(41.06±16.55)세,남녀비례4∶3∶16례(45.7%)환자표현위Goodpasture종합정;18례(51.4%)표현위항GBM신소구신염.24례접수신천자활검환자중,13례(54.2%)표현위신월체신소구신염;7례환자병발기타신소구신염.조Ⅰ,사망7례,50%환자신장장기존활.여조Ⅱ상비,조Ⅰ환자입원시Scr수평、항GBM항체적도、신소구신월체비례균현저승고(P＜0.05);노년환자、빈혈、입원시Scr수평고(＞300μmol/L)급경화신소구비례경고;입원시소뇨혹무뇨、수요혈액투석치료、신장예후차경보편.18례환자적94차DFPP치료중,무명현출혈、저혈압;4례련속동태감측항GBM항체적도적환자중,4～6차DFPP후항GBM항체전음,중위청제솔위55%.결론 근거불동림상표현선택개체화적치료방안유조우개선예후,감소병발증.DFPP능안전유효지청제항GBM항체.
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.
