关键词: idiopathic retroperitoneal fibrosis obstructive renal failure ormond's disease retroperitoneal fibrosis rpf ureteral obstruction ureteral stents

来  源:   DOI:10.7759/cureus.56220   PDF(Pubmed)

Abstract:
Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of fibro-inflammatory tissue surrounding the abdominal aorta and iliac arteries, often leading to the involvement of adjacent organs such as the ureters and inferior vena cava. We present a case report of a 56-year-old Caucasian woman with a complex medical history, including Hodgkin\'s lymphoma treated with chemotherapy and radiotherapy (31 years before), a significant smoking history, and a current presentation of acute kidney injury with oliguria, edema, and hypertension. Initial diagnostic considerations included rapidly progressive glomerulonephritis, supported by clinical and imaging findings. However, the absence of specific autoantibodies and the presence of bilateral calyx-pelvic dilation raised questions regarding alternative diagnoses. Imaging studies, including CT, contrast-enhanced CT, and subsequent MRI, revealed periaortic and paracaval adipose tissue thickening suggestive of periaortitis, leading to a diagnosis of retroperitoneal fibrosis. The multifactorial etiology, including previous radiation therapy, lymphoma history, and significant smoking, posed challenges in establishing a definitive causal link. Despite extensive investigations, including laboratory tests and imaging modalities, no single etiological factor could be conclusively identified. This case underscores the diagnostic complexity of RPF, especially in the presence of multiple potential risk factors, and highlights the importance of considering this condition in the differential diagnosis of patients presenting with renal dysfunction and obstructive uropathy.
摘要:
腹膜后纤维化(RPF)是一种罕见的疾病,其特征是腹主动脉和髂动脉周围存在纤维炎症组织,常导致输尿管和下腔静脉等邻近器官受累。我们提供了一个病例报告,一个56岁的白人妇女有复杂的病史,包括用化疗和放疗治疗的霍奇金淋巴瘤(31年前),有显著的吸烟史,目前出现的急性肾损伤伴少尿,水肿,和高血压。最初的诊断考虑包括快速进展性肾小球肾炎,由临床和影像学检查结果支持。然而,特异性自身抗体的缺失和双侧萼-盆腔扩张的存在引发了关于替代诊断的问题.影像学检查,包括CT,对比增强CT,和随后的MRI,提示主动脉瓣周围和腔旁脂肪组织增厚,导致腹膜后纤维化的诊断。多因素病因,包括以前的放射治疗,淋巴瘤病史,大量吸烟,在建立明确的因果联系方面提出了挑战。尽管进行了广泛的调查,包括实验室测试和成像模式,无法确定单一的病因.这个案例强调了RPF的诊断复杂性,特别是在存在多种潜在风险因素的情况下,并强调了在肾功能不全和阻塞性尿路病变患者的鉴别诊断中考虑这种情况的重要性。
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