以肠梗阻为主要表现的特发性腹膜后纤维化一例并文献复习

摘要

Idiopathic retroperitoneal fibrosis ( RPF) is a rare connective tissue disease with chronic non-specific inflammation in retroperitoneal tissue.Concealed in the early stage , patients with retroperitoneal fibrosis usually don't have symptoms until obstructive nephropathy , renal failure or hypertension happened.Di-agnosis mainly depends on abnormal radiological characteristics.CT and ( or ) MRI are the most commonly used diagnostic methods Whether need biopsy pathological evidence is needed in diagnosis remains controver -sial.In this paper, one, we reparted one case of idiopathic RPF intestinal obstruction as the first magefesta-tion instead of renal failure.RPF was diagnosed via CT inspection of radiography of small intestine.After surgi-cal treatment of ureteroscopy and DJ tube on the right kidney , oral intake of prednisone and tamoxifen , the patient's symptoms had alleviated.This case suggested that vigilance of idiopathic retroperitoneal fibrosis is needed in patient who presented with both intestinal obstruction and urinary tract obstruction , and early diagno-sis and treatment is important for good prognosis.%特发性腹膜后纤维化（ RPF）是以腹膜后组织慢性非特异性炎症为病理特征的一类较少见的结缔组织病。该病早期临床症状隐匿，患者常在发生梗阻性肾病、肾衰竭或高血压后才就诊，诊断主要依赖于影像学资料的异常， CT和（或） MRI是最常采用的诊断手段，关于诊断是否需要行病理活组织检查的病理证据学术界仍有争论。该文分析1例以肠梗阻为首要表现、有泌尿系梗阻但未出现肾衰竭的特发性RPF患者，经CT小肠造影检查明确诊断，行右侧输尿管镜检查加右侧DJ管置入术改善肠道梗阻及泌尿系梗阻，术后给予泼尼松、他莫昔芬，患者病情好转且稳定。因此，临床上对于同时存在肠道梗阻及泌尿系梗阻的患者，需警惕RPF的可能，早期诊断治疗对于患者预后有重要意义。