纤维样肾小球病2例报道及文献复习

A report of 2 cases of fibrillary glomerulopathy and review of literatures

目的通过2例病理确诊为纤维样肾小球病的患者的临床资料分析,结合文献分析,探讨其诊断、治疗方法。方法根据解放军总医院肾脏病研究所收治的2例纤维样肾小球病的患者,结合国内和国外有关治疗效果的报道进行分析。结果 2例病例均为男性,发病年龄从青年到中年,均有肾病水平的蛋白尿,伴或不伴有低白蛋白血症,血压正常或增高,肾功能均正常,未出现急性或慢性肾损伤。病理上,光镜下2例均表现为纤维蛋白样物质在肾小球内的沉积。1例给予肾素-血管紧张素-醛固酮系统(RAAS)阻断剂+激素治疗者部分缓解。1例仅予RAAS阻断剂,未予激素治疗者加重。2例均未出现肾功能损害。结论纤维样肾小球病是一种少见的原发性肾小球疾病,电镜是确诊的必要方法,其预后较差,大多数患者会进展至终末期肾衰。目前对该病尚无明确的治疗方法。

Objective To discuss the method for diagnosis and treatment of fibrillary glomerulopathy through analysis of clinical data of 2 patients in combination with review of related literatures.Methods According to the clinical data of 2 patients admitted in this department,the experience in diagnosis and treatment in combination with review of related literatures was discussed in this article.Results Two patients were all males with age range from youth to middle age,their clinical characteristics manifested as renal proteinuria,with or without hypoalbuminemia,normal or elevated blood pressure and normal renal function,without acute or chronic renal impairment.The pathological findings of 2 cases manifested as fibrin-like material deposition in glomeruli under light microscopic examination.Partial remission was achieved in one case after application of RASS blockers in addition with hormone.The condition of disease worsened in patients without application of hormone therapy.Impairment of renal function had not been occurred in these 2 cases.Conclusion Fibrillary glomerulopathy is a rare primary glomerular disease,electron microscopy is the essential method for its diagnosis,the prognosis of this disease is poor,and most patients will progress to end stage of renal failure.The treatment for this disease is still not clear up to now.