Lioger B, Yahiaoui Y, Kahn J-E, Fakhouri F, Belenfant X, Papo T, Magnant J, Maillot F, Vordos D, Godeau B, Michel M
Service de médecine interne, université Francois-Rabelais, hôpital Bretonneau, CHU de Tours, 2, boulevard Tonnelé, 37044 Tours cedex 9, France.
Service de néphrologie, hôpital du Kremlin-Bicêtre, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France.
Rev Med Interne. 2016 Jun;37(6):387-93. doi: 10.1016/j.revmed.2015.08.013. Epub 2015 Sep 26.
Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the sheathing of retroperitoneal structures by fibro-inflammatory process. It can be either isolated or associated with an underlying disease or condition. In the absence of consistent and consensual approach, the objective of this study was to assess the relevance of diagnostic tests performed during the diagnostic work-up of RPF.
Seventy-seven patients were included in this retrospective multicenter study. The diagnosis of RPF was defined by the presence of a thickened circumferential homogeneous tissue unsheathing the infrarenal aorta, excluding peri-aneurysmal fibrosis and a clear evidence of a cancer.
In 62 cases (80.5%), the RPF was considered as being primary or "idiopathic". Surgical (n=31) or CT-guided (n=9) biopsies of the RPF were performed in half of the patients showing some fibrotic or non-specific inflammatory lesions in 98% of cases. A bone marrow biopsy was performed in 23 patients leading to diagnosis of low grade B cell non-Hodgkin lymphoma in a single patient who also had a monoclonal gammopathy IgM. The systematic search for autoantibodies or serum tumor markers was of no diagnostic value.
Although the diagnostic procedure was heterogeneous, no cause or associated disease was found in the majority of cases of FRP in this series. In the absence of any clinical or paraclinical evidence suggesting an underlying disease or any atypical features at presentation, a number of non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and also more invasive diagnostic tests (bone marrow and RPF biopsies) seem of little relevance.
腹膜后纤维化（RPF）是一种罕见疾病，其特征为纤维炎症过程包裹腹膜后结构。它可以是孤立性的，也可与潜在疾病或病症相关。由于缺乏一致且公认的方法，本研究的目的是评估在RPF诊断检查过程中所进行诊断测试的相关性。
本回顾性多中心研究纳入了77例患者。RPF的诊断定义为存在包绕肾下腹主动脉的增厚的环形均匀组织，排除动脉瘤周围纤维化且无明确癌症证据。
62例（80.5%）患者的RPF被认为是原发性或“特发性”的。一半患者进行了RPF的手术活检（n = 31）或CT引导下活检（n = 9），98%的病例显示出一些纤维化或非特异性炎症病变。23例患者进行了骨髓活检，仅1例同时患有单克隆丙种球蛋白病IgM的患者被诊断为低度B细胞非霍奇金淋巴瘤。系统性检测自身抗体或血清肿瘤标志物无诊断价值。
尽管诊断程序各异，但本系列中大多数RPF病例未发现病因或相关疾病。在没有任何临床或辅助检查证据提示潜在疾病或就诊时无任何非典型特征的情况下，一些非侵入性检查（自身抗体、肿瘤标志物、骨闪烁显像）以及更具侵入性的诊断检查（骨髓和RPF活检）似乎相关性不大。
