特发性腹膜后纤维化7例临床分析  

Clinical analysis of 7 cases of idiopathic retroperitoneal fibrosis

作　　者：冯璐 宋文红 焦媛[1] FENG Lu;SONG Wenhong;JIAO Yuan(Department of Rheumatology and Immunology,Liaoning Healthy Group BenGang General Hospital,Benxi 117000,China)

机构地区：[1]辽健集团本钢总医院风湿免疫科,辽宁本溪117000

出　　处：《大连医科大学学报》2020年第6期533-536,共4页Journal of Dalian Medical University

摘　　要：目的探讨特发性腹膜后纤维化(idilpathic retroperitoneal fibrosis,RPF)患者的临床特点。方法回顾性分析2015—2019年在辽健集团本钢总医院风湿免疫科住院并诊断为RPF 7例患者的临床资料。其中男性7例,女性1例。4例患者行输尿管支架术,所有患者均加用糖皮质激素及免疫抑制剂治疗。对7例患者的临床表现、血清学相关指标、影像学检查及疗效进行分析。结果 1例患者反复低热,4例有腹痛等临床症状,5例肾功能不全。7例患者行常规CT检查均呈现腹膜后密度均匀的软组织肿块影,包绕腹主动脉、下腔静脉、输尿管,病变位置与其周围组织没有清楚的界限。其中6例行CT扫描增强后,轻中度强化5例,无明显强化1例。治疗前7例患者ESR、CRP、IgG4、Cr、BUN、i-PTH、CysC均值均高于正常,治疗3个月后上述指标均较治疗前下降,差异均有统计学意义,P<0.05;复查腹部CT发现腹膜后软组织肿块影范围较前均减小。结论 RPF临床表现无明显特异性,腹部影像学检查发现腹主动脉、下腔静脉、髂动静脉、输尿管等周围存在包块或包裹可协助诊断,必要时行组织活检明确诊断。激素及免疫抑制剂治疗对该病有效,预后较好。Objective To investigate clinical characteristics of patients with idiopathic retroperitoneal fibrosis(RPF).Methods The clinical data of 7 patients with RPF were retrospectively analyzed(male 6,female 1).Among them,4 patients recieved ureteric sent placement,7 patients all recieved treatment with glucocorticoid combined with immunosuppressive agents.The clinical manifestations,auxiliary examinations,treatment options and curative effects were analyzed.Results Fever was observed in 1 case,abdominal pain in 4 cases,renal insufficiency in 5 cases.CT showed that the RPF had a soft tissue shadow or lump in the retroperitoneal space.Contrast-enhanced CT was performed in 6 cases and revealed mild and moderate enhancement in 5 cases and no reinforcement in 1 case.The ESR,CRP,IgG4,Cr,BUN,i-PTH,and CysC levels were higher than normal before treatment,and significantly decreased after 3 months of treatment.Follow-up CT revealed that the size of PRF became smaller significantly.Conclusions RPF has no typical clinical manifestations.CT and MRI examination may reveal abnormal density lesions surrounding large retroperitoneal blood vessels.Biopsy should be performed to confirm the diagnosis.Treatment with glucocorticoid combined with immunosuppressive agents is effective.

关 键 词：原发性腹膜后纤维化 临床特点 糖皮质激素 免疫抑制剂 

分 类 号：R593.2[医药卫生—内科学]