儿童肾病综合征型狼疮性肾炎30例临床、病理及近期疗效观察

Clinics,pathology and prognosis of 30 children with nephrotic syndrome in clinical trials of lupus nephritis

目的总结儿童肾病综合征(NS)型狼疮性肾炎(LN)的临床、病理特点,分析治疗方案与近期疗效,为NS型LN患儿的诊治提供参考.方法收集2009年1月1日至2018年12月31日确诊为NS型LN患儿的临床资料,进行回顾性分析.结果107例LN患儿中,NS型共30例,占28.0%.发病年龄5~13岁,平均(9.7±2.3)岁.其中男6例,女24例,男女比例为1:4.最常见的肾外表现为贫血.与非NS型LN患儿相比,NS型LN患儿合并急性肾损伤与高血压比例高、白细胞减少比例低(P<0.001;P<0.001;P=0.01).行肾穿刺活检22例,最常见的病理类型为Ⅳ型,共12例(54.5%),其次为Ⅴ型5例(22.7%)、Ⅱ型3例(13.6%)、Ⅴ+Ⅱ型与Ⅴ+Ⅳ型各1例(4.5%).30例患儿均给予泼尼松联合免疫抑制剂治疗,其中大剂量甲泼尼龙(MMP)联合环磷酰胺(CTX)冲击19例(63.3%),MMP冲击后序贯吗替麦考酚酯(MMF)1例(3.3%),仅用CTX冲击3例(10.0%),MMF联合他克莫司2例(6.7%),仅MMF 1例(3.3%),用药不依从4例(13.3%).治疗6个月,完全缓解17例,部分缓解8例,未缓解4例,死亡1例,死亡原因为合并重症肺炎与严重脓毒症.结论NS型LN患儿合并急性肾损伤与高血压比例高于非NS型,病理类型具有多样性,弥漫增生性Ⅳ型是主要的病理类型.MMP联合CTX冲击是临床表现为NS型或病理表现为Ⅳ型LN患儿主要的诱导治疗方案,可有效诱导缓解.

Objective To summarize the clinical and pathological characteristics of children with nephrotic syndrome(NS)in clinical trials of lupus nephritis(LN),analyze the treatment plan and prognosis,and provide reference for the diagnosis and treatment.Methods Clinical data of children diagnosed with NS-type LN were collected for retrospective analysis from January 1,2009 to December 31,2018.Results NS was found in 30 cases(28.0%)of 107 cases with LN.The ages of onset were 5-13 years,average(9.7±2.3)years.Among them,there were 6 males and 24 females with a male to female ratio of 1∶4.The most common extrarenal manifestation is anemia.Compared with non-NS-type LN patients,the proportion of acute kidney injury and hypertension was higher and the proportion of leukopenia was lower in NS-type LN patients(P<0.001;P<0.001;P=0.01).Renal biopsy showed the most common is classⅣ(54.5%),followed by classⅤ(22.7%),Ⅱ(13.6%),Ⅴ+Ⅱ(4.5%)andⅤ+Ⅳ(4.5%)in 22 cases.Thirty cases were given prednisone plus immunosuppressant,including intravenous methylprednisolone pulses(MMP)combined with cyclophosphamide(CTX)(63.3%),sequential usage of mycophenolate mofetil(MMF)after MMP(3.3%),only with CTX(10.0%),MMF combined with tacrolimus(6.7%),only with MMF(3.3%),and poor medication compliance(13.3%).After 6 months of treatment,17 cases had complete remission,8 cases had partial remission,4 cases had no remission,and 1 case had died of severe pneumonia and severe sepsis.Conclusion The proportion of acute kidney injury and high blood pressure in NS-type LN patients is high than those in non-NS-type LN patients.In NS-type LN patients,the renal pathology is complex,and classⅣtype is the main pathological type.Among the patients with NS-type LN or pathological type ofⅣ,MMP joint CTX is the main induction therapy,which can effectively induce remission.