慢性主动脉周围炎患者临床特征分析及鹅脱氧胆酸在其发病机制中的临床意义

Clinical analysis of chronic periaortitis and the clinical significance of chenodeoxycholic acid in the pathogenesis of chronic periaortitis

目的分析慢性主动脉周围炎(chronic periaortitis,CP)患者的临床特征,初步探索鹅脱氧胆酸(chenodeoxycholic acid,CDCA)在CP发病机制中的意义,为治疗该病提供新思路。方法回顾性分析2004年10月至2017年3月期间17例CP患者的临床资料。液相色谱-串联质谱法(UPLC-MS/MS)检测5例CP患者及各对照组患者血清中CDCA含量。此外,对1例活动期CP患者进行后腹膜穿刺,病理标本行免疫组化检测IL-6、巨噬细胞表面特异性标志物F4/80及法尼酯受体(farnesoid-X-receptor,FXR)表达情况。结果 17例CP患者中,男女比例为3.25∶1,平均年龄为(62.5±8.4)岁。患者的首发症状有腰痛、腹痛等。实验室检查可有血沉和C反应蛋白的升高。与各对照组患者相比,CP患者血清中CDCA含量明显降低。免疫组化结果显示,脂肪和胶原纤维组织内泡沫细胞样细胞和淋巴细胞聚集部位可见IL-6高表达,并有F4/80及FXR的表达。结论 CP是一种罕见的自身免疫性炎症性疾病,起病隐匿,临床特征各异。血清中CDCA降低可能是该疾病的新临床指标。CDCA-FXR/IL-6通路可能在CP发病中起到一定作用。

Chronic periaortitis （CP） is a rare autoimmune disease and serum IL-6 level was found significantly elevated in active CP patients. This study was undertaken to investigate the clinical characteristics of CP and preliminarily explore the role of chenodeoxycholic acid （CDCA） in the pathogenesis of the disease. The clinical data of 17 CP patients, diagnosed from October 2004 to March 2017, were analyzed retrospectively. Serum CDCA levels of 5 CP patients and controls were detected by ultra performance liquid chromatography-tandem mass spectrometry （UPLC-MS/MS） method. The pathological specimen obtained by retroperitoneal puncture from 1 CP patient was stained by immunohistochemistry to detect the expression of IL-6, surface-specific marker of macrophage F4/80 and farnesoid-X-receptor （FXR）. As to 17 CP patients, male： female is 3.25 ： 1 and the average age of onset is 62.5± 8.4 years. Primary symptoms included abdominal pain, flank and back pain and so on. ESR and CRP could also be elevated. Compared with each control group, serum CDCA levels were significantly reduced in CP patients. Immunohistochemical results showed that IL-6 was highly expressed in the aggregation sites of foam-like cells and lymphocytes within the adipose tissues and collagen fibers. F4/80 and FXR were also expressed. In conclusion.reduced CDCA expression in serum may be a new clinical index of this disease and CDCA-FXR/IL-6 pathway may play a role in the pathogenesis of CP.