腹膜后纤维化7例的临床特点和治疗方案分析

发布时间：2019-04-04 19:19

【摘要】：目的分析原发性腹膜后纤维化(RPF)患者的临床特点、治疗方法和预后,以提高对该疾病的认识。方法回顾性分析2011—2016年在上海交通大学医学院附属瑞金医院肾脏科住院并被诊断为原发性RPF的7例患者首诊时的临床表现、实验室检查、影像学检查、治疗方案和预后等资料。结果 7例患者中,男6例、女1例;中位发病年龄为55岁;起病时出现多饮、多尿症状1例,腰腹疼痛2例,无任何症状但在体格检查时发现异常4例。实验室检查结果示,6例出现不同程度的肾功能不全、CRP水平升高;7例红细胞沉降率升高。7例患者均进行泌尿系统CT或MRI检查,可见软组织影包绕腹主动脉、髂动脉、输尿管,部分患者甚至包绕肾动静脉或腹膜后其他组织,均符合RPF的诊断;其中4例行腹膜后肿块活组织病理学检查,结果均提示纤维组织增生,伴有淋巴细胞和浆细胞浸润。3例因泌尿系统梗阻行双J管植入术,1例行肾盂造瘘术,余3例均未进行外科干预;4例因血肌酐水平升高明显而行中心静脉插管血液透析治疗;1例采用单纯糖皮质激素治疗,6例采用糖皮质激素联合免疫抑制剂治疗(1例先后采用糖皮质激素联合吗替麦考酚酯、环磷酰胺、帕夫林等治疗,但因疗效不佳且伴有激素依赖而加用利妥昔单抗治疗;余5例均采用糖皮质激素联合环磷酰胺治疗)。随访3~56个月,中位随访时间为23.0个月。1例因环磷酰胺治疗后骨髓抑制导致感染而死亡,其余患者未再出现梗阻症状,无患者进展至终末期肾功能不全;其中因激素依赖、联合多种免疫抑制剂治疗无效、疾病反复复发而予利妥昔单抗治疗的1例患者,在随访过程中逐步减少泼尼松用量后,患者肾功能稳定,且无过敏、感染等并发症发生。结论 RPF的临床表现无明显特异性,影像学检查对诊断的价值较高。外科与内科结合治疗、糖皮质激素联合免疫抑制剂治疗是RPF的常用治疗方案,对于激素依赖、联合多种免疫抑制剂治疗无效、疾病反复复发的患者可予利妥昔单抗治疗,但其疗效与安全性尚需进一步研究予以证实。
[Abstract]:Objective to analyze the clinical characteristics, treatment and prognosis of patients with primary retroperitoneal fibrosis (RPF) in order to improve the understanding of the disease. Methods from 2011 to 2016, 7 patients who were hospitalized in Ruijin Hospital of Medical College of Shanghai Jiaotong University and diagnosed as primary RPF were analyzed retrospectively on their clinical manifestations, laboratory examination and imaging examination. Treatment plan and prognosis. Results among the 7 patients, 6 were male and 1 female, the median age of onset was 55 years, polyuria occurred in 1 case, abdominal pain in 2 cases, no symptoms were found in 4 cases but abnormal in physical examination. The results of laboratory examination showed that 6 cases had different degree of renal insufficiency and the level of CRP increased. Seven patients were examined by CT or MRI in the urinary system. Soft tissue enveloping abdominal aorta, iliac artery, ureter, and even renal arteriovenous or retroperitoneal tissue were found in 7 patients. All of them were in accordance with the diagnosis of RPF. Among them, 4 cases underwent pathological examination of retroperitoneal mass, the results showed that fibrous tissue hyperplasia was accompanied by infiltration of lymphocytes and plasmacytes, 3 cases underwent double J tube implantation due to urinary obstruction, 1 case underwent pyelostomy, 1 case underwent pyelostomy, and 1 case underwent pyelostomy. No surgical intervention was carried out in the other 3 cases. 4 cases were treated with hemodialysis by central venous catheterization because of the obvious increase of serum creatinine level. One case was treated with glucocorticoid alone, 6 cases were treated with glucocorticoid combined with immunosuppressive agents (1 case was treated with corticosteroid combined with mexicol ester, cyclophosphamide, Pavlin, etc.), and 1 case was treated with corticosteroids combined with immunosuppressive agents. However, Rituximab was used in addition to Rituximab because of poor curative effect and hormone dependence. The other 5 cases were treated with glucocorticoid combined with cyclophosphamide. The median follow-up time was 23.0 months. One patient died of infection caused by myelosuppression after cyclophosphamide treatment, and the other patients had no obstruction symptoms and no progression to end-stage renal failure. One patient was treated with rituximab because of hormone dependence, combined with multiple immunosuppressive agents and repeated recurrence of the disease. After gradually reducing the dosage of prednisone during the follow-up, the renal function of the patient was stable and there was no allergy. Complications such as infection occur. Conclusion the clinical manifestation of RPF has no obvious specificity, and imaging examination is of great value in diagnosis. Surgery combined with internal medicine, glucocorticoid combined with immunosuppressive therapy is a common treatment for RPF, for hormone dependence, combined with multiple immunosuppressive therapy, patients with recurrent disease can be treated with rituximab. But its curative effect and safety need further research to confirm.
【作者单位】：上海交通大学医学院附属瑞金医院肾脏科;上海交通大学医学院附属瑞金医院放射科;
【分类号】：R572.2

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