通讯作者: 林燕, liny7154@163.com

DOI：10.12201/bmr.202410.00077

Exploration of Diagnostic and Treatment Experience in Two Cases of Idiopathic Retroperitoneal Fibrosis

• 摘要：目的 报道两例特发性腹膜后纤维化的病例,并结合文献复习,以增进对该病的认识,提高临床对该病的敏感性。方法 报道了1名71岁女性及1名68岁男性2位特发性腹膜后纤维化患者的病例,分别从临床表现、实验室检查、影像学表现及治疗方法等方面进行介绍。同时,结合文献复习该病的发病机制、诊断和治疗方法。结果 2例患者主要表现为水肿、乏力等非特异性表现,并由于输尿管受压出现肾脏病相关表现。影像学检查显示输尿管和肾脏受压。2例患者分别采用激素及输尿管支架置入术治疗,症状和影像学表现均得到改善,短期效应良好。文献复习表明,特发性腹膜后纤维化是一种罕见自身免疫性疾病,临床表现缺乏特异性,目前确诊依赖影像学及组织学检查。现有临床治疗方式主要包括药物及手术治疗。结论 特发性腹膜后纤维化临床表现不典型,常易被忽视。临床医生应提高对该病的认识,注意不明原因的腰痛、输尿管梗阻等表现,并尽早采取激素等治疗措施。未来研究应集中在特异性诊断手段和更有效的治疗制剂上,以提高早期诊断率和治疗效果。

  关键词： 特发性腹膜后纤维化; 自身免疫性疾病; 诊治; 输尿管支架置入术; 病例报告

   

  Abstract: Objective To report two cases of idiopathic retroperitoneal fibrosis and review the literature to enhance understanding and clinical alertness to this condition. Methods We present the cases of two patients with idiopathic retroperitoneal fibrosis, a 71-year-old woman and a 68-year-old man, detailing their clinical presentations, laboratory findings, imaging characteristics, and treatment approaches. We also review the literature to discuss the pathogenesis, diagnostic strategies, and treatment options for this disease. Results Both patients exhibited nonspecific symptoms, such as edema and fatigue, along with renal-related symptoms due to ureteral compression. Imaging revealed compression of the ureters and kidneys. Treatment with corticosteroids and ureteral stent placement led to improvements in symptoms and imaging findings, demonstrating favorable short-term outcomes. The literature review highlights that idiopathic retroperitoneal fibrosis is a rare autoimmune disorder with nonspecific clinical features, and diagnosis is primarily based on imaging and histopathological evaluations. Current treatment modalities include both pharmacological and surgical interventions. Conclusion Idiopathic retroperitoneal fibrosis presents with non-specific clinical symptoms and can be easily missed. Clinicians should be more vigilant about this condition, considering it in the differential diagnosis of unexplained back pain or ureteral obstruction, and initiating timely treatment with corticosteroids, among other interventions. Future research should focus on developing specific diagnostic tools and more effective therapeutic agents to enhance early detection and treatment efficacy.

  Key words: ; ; ; ; 

  提交时间：2024-10-31

  版权声明：作者本人独立拥有该论文的版权，预印本系统仅拥有论文的永久保存权利。任何人未经允许不得重复使用。
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• 序号	提交日期	编号	操作
  1	2024-08-04	bmr.202410.00077V1	下载

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