Rodríguez Jornet A, Andreu Navarro F J, Orellana Fernández R, Ibeas López J, García García M
Servicio de Nefrología, Corporación Sanitaria Parc Taulí, Sabadell.
Nefrologia. 2009;29(4):298-303. doi: 10.3265/Nefrologia.2009.29.4.5344.en.full.
Idiopathic retroperitoneal fibrosis is a rare disease often causing obstructive uropathy because the fibrosis entraps the ureters. The retroperitoneal tissue is constituted by a fibrous component and a chronic inflammatory infiltrate with the former characterized by miofibroblasts. The infiltrate displayed perivascular. Last years, the immunosuppressive therapy and the decompression of obstructive renal failure have improved the prognostic. We reported 9 patients with Idiopathic retroperitoneal fibrosis in a Centre. We evaluate clinical symptoms at the presentation with the signs of renal and non-renal involvement. We evaluate the achieved therapy and the follow-up. All 9 patients had a radiological and/or histological diagnosis in the absence of malignancy, previous radiotherapy or gadolinium's administration with severe renal failure. The patients were treated with 1 mg/kg/day of prednisone three months and possibly the insertion of ureteral catheters. 89% patients have a high rate of initial success without renal failure, despite frequent disease relapse. A patient developed progression of the fibrosis.
特发性腹膜后纤维化是一种罕见疾病，常因纤维化包绕输尿管而导致梗阻性尿路病。腹膜后组织由纤维成分和慢性炎性浸润构成，前者以肌成纤维细胞为特征。炎性浸润呈血管周围性。近年来，免疫抑制治疗和梗阻性肾衰竭减压改善了预后。我们在一个中心报告了9例特发性腹膜后纤维化患者。我们评估了就诊时的临床症状以及肾脏和非肾脏受累的体征。我们评估了所采用的治疗方法及随访情况。所有9例患者均在无恶性肿瘤、既往放疗或使用钆且伴有严重肾衰竭的情况下获得了放射学和/或组织学诊断。患者接受了为期三个月的每日1毫克/千克泼尼松治疗，并可能置入输尿管导管。89%的患者初始成功率较高且无肾衰竭，尽管疾病频繁复发。有1例患者出现了纤维化进展。
