摘要
目的了解冷球蛋白血症相关肾小球肾炎(cryoglobulinemic glomerulonephritis,CGN)临床病理特点及治疗预后。方法回顾性总结分析郑州大学第一附属医院肾内科2015年4月至2019年1月诊断为冷球蛋白血症相关肾小球肾炎患者的临床病理特点及治疗预后。结果共入选22例患者,平均年龄(44.1±15.1)岁,男女比例6.3∶1。12例伴HBV感染(54.5%),5例伴单克隆免疫球蛋白血症(monoclonal immunoglobulin,MIg)。肾病范围蛋白尿和镜下血尿患者占90.1%、54.5%,膜增生性肾小球肾炎(MPGN,72.7%)最多见。平均随访时间(15.8±11.9)个月,总缓解14例(63%),无应答5例(22.7%),死亡2例(9.1%),ESRD1例(4.5%)。CGN伴HBV感染患者均给予血管紧张素受体拮抗剂(ARB)类联合抗病毒药物治疗,其中6例(50%)后期加用糖皮质GC或联合免疫抑制剂,总缓解率75%;1例MGRS相关CGN患者给予地塞米松联合硼替佐米方案治疗1周期后完全缓解。结论冷球蛋白血症相关肾小球肾炎临床多表现为肾病综合征,肾脏病理常为膜增生性肾小球肾炎,国内可能与HBV感染相关较多见,治疗方案应根据病因及临床病理表现合理选择。
Objective To elucidate the clinicopathological features and prognosis of the patients with cryoglobulinemic glomerulonephritis.Methods In this retrospective study,the clinicopathological features and prognosis of patients diagnosed as cryoglobulinemic glomerulonephritis in the first affiliated hospital of Zhengzhou university during the period between April 2015 and January 2019 were analyzed.Results Altogether 22 patients were included with an average age of(44.1±15.1)years old,male/female ratio was 6.3∶1.There were 12 patients with infection of HBV(54.5%),5 patients with monoclonal immunoglobulin(MIg).Nephrotic proteinuria presented in 90.1%of cases,and microscopic hematuria in 54.5%.Membrano-proliferative glomerulonephritis(MPGN,72.7%)was the predominant pathological type.The patients had a mean follow-up of(15.8±11.9)months.14 patients(63.6%)achieved complete/partial remission,5 patients(22.7%)with no response,2 cases died(9.1%),1 case(4.5%)progressed to ESRD.CGN patients with infection of HBV were all treated with ARB plus antiviral drug,and 6 cases added glucocorticoid and immunosuppressor later,75%patients achieved remission.One MGRS associated CGN patient treated with dexamethasone and bortezomib,achieved complete remission after 1 cycle.Conclusion The main clinicopathological features of CGN was nephrotic syndrome and MPGN.Domestic patients maybe more associated with HBV infection.Treatment recommendations differ depending on etiology and clinicopathological features.
作者
卢珊
董奕君
张艳
LU Shan;DONG Yi-jun;ZHANG Yan(Department of Nephrology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)
出处
《医药论坛杂志》
2021年第23期26-29,34,共5页
Journal of Medical Forum
基金
河南省科技攻关计划项目(212102310772)
关键词
冷球蛋白血症
肾小球肾炎
疾病特点
预后
Cryoglobulinemia
Kidney disease
Disease characteristics
Prognosis
