腹膜后纤维化33例临床分析并文献复习  

Retroperitoneal fibrosis：a clinical and outcome analysis of 33 cases and review of literature

作　　者：刘航[1] 肖卫国[1] 

机构地区：[1]中国医科大学附属第一医院风湿免疫科,沈阳110001

出　　处：《中华风湿病学杂志》2013年第6期383-386,共4页Chinese Journal of Rheumatology

摘　　要：目的探讨腹膜后纤维化（RPF）临床特点及转归。方法对经住院确诊的33例RPF患者临床资料和转归进行回顾分析，并复习国内外文献。结果RPF中老年男性较女性多见，腰痛、腹痛及下肢水肿是最常见的临床表现。实验室检查以炎性指标红细胞沉降率及c反应蛋白升高明显，压迫输尿管患者伴有肾功能不全。所有患者均行CT或磁共振成像检查见腹膜后软组织密度影，其中4例行病理活组织检查确定诊断。23例行手术解除梗阻治疗；18例行内科激素及免疫抑制剂系统治疗；9例行内外科结合治疗。手术联合内科规律治疗预后良好。结论影像学检查具有重要价值，确诊需病理检查，长期维持小剂量激素及免疫抑制剂对治疗有效并可减少复发。Objective To investigate the clinical features and outcome of retroperitoneal fibrosis （RPF）. Methods Thirty-three cases of RPF treated in the Department of Rheumatology and Immunology were analyzed retrospectively, including the clinical characteristics and laboratory data. Results RPF occurred predominantly in elderly men with untypical clinical manifestations such as back pain, abdominal pain and lower limb edema. In laboratory tests, the inflammatory parameters such as erythrocyte sedimentation rate and C-reactive protein increased significantly, and renal function failure was frequent finding in patients with urethral obstruction. All patients had retroperitoneal soft tissue image or urethral obstruction on computed tomography or magnetic resonance imaging, four of which had histological diagnosis of idiopathic RPF. Twenty-three patients received surgical interventions. Eighteen patients received medical treatment alone including eorticosteroids, immunosuppressants, and tamoxifen. Nine patients received corticosteroids after surgical intervention. Surgery and medical treatments are effective for idiopathic RPF. Conclusion Image examination is valuable in diagnosis and differential diagnosis of PRF. Pathological diagnosis is the golden standard. Long-term low dose corticosteroids and immunosuppressants may prevent relapse of idiopathic RPF.

关 键 词：腹膜后纤维化 治疗 IGG4相关性疾病 

分 类 号：R572.2[医药卫生—消化系统]