CAJ | 학술논문

目的探讨RAS抑制剂在病理诊断的狼疮肾炎(LN)伴血栓性微血管病(TMA)治疗中的作用及对预后的影响.方法选取2000年1月至2013年12月期间在北京协和医院住院,肾活检病理诊断为LN合并TMA,并接受RAS抑制剂治疗的患者,共入选15例.回顾性分析应用RAS抑制剂前后患者Scr、血压的变化.结果 (1)男女比1∶14,所有患者入院时均出现肾功能不全,Scr峰值中位数为396 μmol/L(160～ 643 μmol/L),5例(33.3％)患者接受临时透析治疗,高血压发生率100％,其中恶性高血压6例(40.0％).(2)所有患者均出现贫血,14例出现血小板减少,其中确诊和疑诊微血管病性溶血性贫血(MAHA)的分别为3例(20.0％)和5例(33.3％).(3)LN病理类型:Ⅱ型1例,Ⅲ型4例,Ⅳ(G)型2例,Ⅳ(S)型5例,Ⅳ+Ⅴ型3例.肾小球病变以活动性病变为主,TMA样病变以急性期改变为主.(4)患者在应用RAS抑制剂前均接受糖皮质激素及免疫抑制剂治疗,其中9例接受糖皮质激素冲击治疗,13例同时接受环磷酰胺(CTX)治疗,其余2例先后应用环磷酰胺、霉酚酸酯(MMF).5例(55.6％)患者激素冲击治疗后Scr降低.13例(86.7％)患者在应用RAS抑制剂1周内平均动脉压(MAP)降低幅度为15.8％(9.4％～29.7％),Scr降低幅度为17.0％(9.7％～40.0％),其余2例因合并其他原因血压和Scr升高.(5)4例患者失访,其余11例患者中位随访时间为32个月(8～135个月).5例临时透析患者均脱离透析,其余患者肾功能稳定或进一步好转.结论对于临床表现为急性肾损伤、难治性高血压的LN伴TMA患者,在免疫抑制治疗的基础上,应考虑使用RAS抑制剂,可能使临时透析患者脱离透析,进而改善肾脏远期预后.
목적 탐토RAS억제제재병리진단적랑창신염(LN)반혈전성미혈관병(TMA)치료중적작용급대예후적영향.방법 선취2000년1월지2013년12월기간재북경협화의원주원,신활검병리진단위LN합병TMA,병접수RAS억제제치료적환자,공입선15례.회고성분석응용RAS억제제전후환자Scr、혈압적변화.결과 (1)남녀비1∶14,소유환자입원시균출현신공능불전,Scr봉치중위수위396 μmol/L(160～ 643 μmol/L),5례(33.3％)환자접수림시투석치료,고혈압발생솔100％,기중악성고혈압6례(40.0％).(2)소유환자균출현빈혈,14례출현혈소판감소,기중학진화의진미혈관병성용혈성빈혈(MAHA)적분별위3례(20.0％)화5례(33.3％).(3)LN병리류형:Ⅱ형1례,Ⅲ형4례,Ⅳ(G)형2례,Ⅳ(S)형5례,Ⅳ+Ⅴ형3례.신소구병변이활동성병변위주,TMA양병변이급성기개변위주.(4)환자재응용RAS억제제전균접수당피질격소급면역억제제치료,기중9례접수당피질격소충격치료,13례동시접수배린선알(CTX)치료,기여2례선후응용배린선알、매분산지(MMF).5례(55.6％)환자격소충격치료후Scr강저.13례(86.7％)환자재응용RAS억제제1주내평균동맥압(MAP)강저폭도위15.8％(9.4％～29.7％),Scr강저폭도위17.0％(9.7％～40.0％),기여2례인합병기타원인혈압화Scr승고.(5)4례환자실방,기여11례환자중위수방시간위32개월(8～135개월).5례림시투석환자균탈리투석,기여환자신공능은정혹진일보호전.결론 대우림상표현위급성신손상、난치성고혈압적LN반TMA환자,재면역억제치료적기출상,응고필사용RAS억제제,가능사림시투석환자탈리투석,진이개선신장원기예후.
Objective To investigate whether RAS inhibitors can improve renal function in the treatment of lupus nephritis (LN) with thrombotic microangiopathy (TMA).Methods A total of 15 LN patients with TMA proven by renal pathology,from January 2000 to December 2013 in PUMCH,were included.The serum creatinine (Scr) and blood pressure (BP) before and after using RAS inhibitors were analyzed.Results (1)Male/female ratio was 1∶ 14.All of the patients had renal dysfunction,and median peak value of Scr was 396 μmol/L (160 ～ 643 μmol/L).5 cases (33.3％) required acute dialysis during hospitalization.Hypertension occurred in 15 patients,while 6 cases (40.0％) were diagnosed malignant hypertension.(2) Anemia and thrombocytopenia occurred in 15 and 14 cases,respectively.Three cases (20.0％) were diagnosed MAHA definitely and 5 cases (33.3％) were diagnosed MAHA probably.(3) Renal biopsy showed class Ⅱ in 1 case,Ⅲ in 4 cases,Ⅳ-(G) in 2 cases,Ⅳ(S) in 5 cases and Ⅳ+Ⅴ in 3 cases.Active lesions were predominant in both glomeruli and renal vasculopathy.(4) All the patients received steroid and immunosuppressive therapy,of whom 9 cases were given steroid pulse therapy.Thirteen cases received cyclophosphamide,and the rest 2 cases received cyclophosphamide and mycophenolate.After steroid pulse therapy,there were only 5 patients (55.6％) who got decreased Scr.In 13 patients (86.7％),hypertension was ameliorated and Scr decreased within one week after implementing RAS inhibitors,which fell medianly 15.8％ and 17.0％,respectively.(5) Eleven of the 15 patients were followed from 8 to 135 months (median 32 months),and the other 4 patients were lost.Five cases who was on dialysis during hospitalization became independent of renal replacement therapy,while the other cases also got improved renal function.Conclusions Patients of LN with TMA who develop AKI and refractory hypertension should be treated with RAS inhibitors.Improved renal survival and successful discontinuation of dialysis are possible benefits when RAS inhibitors are used to treat LN with TMA.