Urology and Nephrology, San Luigi Gonzaga University Hospital, Regione Gonzole 10, Orbassano Torino, Turin, Italy.
Nephrol Dial Transplant. 2010 Aug;25(8):2603-10. doi: 10.1093/ndt/gfq051. Epub 2010 Feb 15.
Retroperitoneal fibrosis (RF) is a complex clinical entity characterized by a fibro-inflammatory reaction around the abdominal aorta and iliac arteries extended into the retroperitoneum. No biochemical marker correlates with the disease severity and progression, and imaging data fail to discriminate between fibrotic and florid lesions. Positron emission tomography (PET) was recently suggested as a promising tool to detect the disease.
We report on seven consecutive cases of RF managed by tailoring therapeutic interventions to the metabolic activity detected by PET. In 2006-09, seven patients with RF (five new diagnoses) were referred to the same nephro-urological facility. There were six males and one female aged 41-79. RF was associated with autoimmune diseases in three patients, with an aortic aneurysm in another three, and was 'idiopathic' in one. The diagnoses were made by imaging techniques [computed tomography (CT) or nuclear magnetic resonance (NMR)]; PET scan was performed in all patients in the same setting at referral and during follow-up.
Patients were followed up with tailored interventions (medical therapy: tamoxifen, steroids, and immunosuppressors according to disease activity, side effects and tolerance). Six patients needed ureteral stenting for obstruction. PET imaging was used as a guide for the tapering of immunosuppressors and for stent removal. In this way, stents were safely removed when a negativization of disease activity was revealed by PET. Only one relapse was recorded over 163 months of follow-up (median 24 months) detected in time by PET.
PET is a promising tool for surveillance of disease activity and for planning the removal of ureteral stents in RF.
腹膜后纤维化（RF）是一种复杂的临床实体，其特征是腹主动脉和髂动脉周围发生纤维炎症反应，并延伸至腹膜后腔。目前没有与疾病严重程度和进展相关的生化标志物，影像学数据也无法区分纤维性和活动性病变。正电子发射断层扫描（PET）最近被认为是一种有前途的检测疾病的工具。
我们报告了 7 例 RF 患者，通过将治疗干预措施与 PET 检测到的代谢活性相匹配来管理这些患者。2006 年至 2009 年，7 例 RF 患者（5 例新诊断）被转诊到同一肾病泌尿科机构。其中 6 例为男性，1 例为女性，年龄为 41-79 岁。3 例 RF 与自身免疫性疾病相关，3 例与主动脉瘤相关，1 例为“特发性”。诊断通过影像学技术（计算机断层扫描（CT）或磁共振成像（NMR））进行；所有患者在转诊时和随访期间在同一环境中进行 PET 扫描。
患者接受了针对性干预（根据疾病活动、副作用和耐受性，采用他莫昔芬、类固醇和免疫抑制剂进行治疗）。6 例患者因梗阻需要输尿管支架置入。PET 成像用于指导免疫抑制剂的减量和支架的移除。在这种情况下，当 PET 显示疾病活动阴性时，安全地移除了支架。在 163 个月（中位数 24 个月）的随访中仅记录到 1 例复发，及时通过 PET 检测到。
PET 是监测疾病活动和计划 RF 患者输尿管支架移除的一种很有前途的工具。
