Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by ongoing inflammatory destruction of the interlobular bile ducts, eventually leading to chronic cholestasis and biliary cirrhosis. This study primarily aims to define the metabolomic signature of PBC after comparison with healthy controls (HC). Second, it aims to evaluate the possible metabolic association between PBC and celiac disease (CD), an immune-mediated disorder frequently associated with PBC. Serum and urine samples from 20 PBC, 21 CD, and 19 sex-matched HC subjects were collected. 1H nuclear magnetic resonance (NMR) spectra for all samples were acquired, and multivariate statistics were used to evaluate the differences among the three groups and to provide information about the involved metabolites. The classification accuracies to discriminate PBC and HC groups were 78.9-84.6% for serum and 76.9% for urine. In comparison to HC, PBC patient sera were characterized by altered levels ( p value <0.05) of pyruvate, citrate, glutamate, glutamine, serine, tyrosine, phenylalanine, and lactate. PBC patient urine showed lower levels ( p value <0.05) of trigonelline and hippurate with respect to HC. Furthermore, the NMR metabolomic fingerprint was able to cluster PBC with respect to CD patients, and the classification accuracies in the discriminations between these groups were 81.9-91.7% for serum and 77.7% for urine. Our results show that PBC displays a unique metabolomic fingerprint, which led to speculation about an impaired energy metabolism, probably associated with an altered gut microbiota. PBC and CD showed two distinct metabolic fingerprints. These data could provide clues for the comprehension of the PBC pathogenetic mechanisms and the detection of novel therapeutic targets.

中文翻译：

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原发性胆源性胆管炎的代谢特征及其与乳糜泻的比较。

原发性胆源性胆管炎（PBC）是一种慢性自身免疫性肝病，其特征是小叶间胆管持续发生炎性破坏，最终导致慢性胆汁淤积和胆汁性肝硬化。这项研究的主要目的是在与健康对照（HC）比较后确定PBC的代谢组学特征。其次，其目的是评估PBC与腹腔疾病（CD）之间的可能的代谢关联，该疾病通常是与PBC相关的免疫介导的疾病。收集了20位PBC，21位CD和19位性别匹配的HC受试者的血清和尿液样本。获得了所有样品的1H核磁共振（NMR）光谱，并使用多元统计量评估了三组之间的差异并提供了有关所涉及代谢物的信息。区分PBC和HC组的分类准确度分别为血清78.9-84.6％和尿液76.9％。与HC相比，PBC患者血清的特征在于丙酮酸，柠檬酸，谷氨酸，谷氨酰胺，丝氨酸，酪氨酸，苯丙氨酸和乳酸水平的改变（p值<0.05）。PBC患者尿液相对于HC而言，曲安奈林和马尿酸盐的水平较低（p值<0.05）。此外，NMR代谢组学指纹能够使CD患者聚集在PBC上，这些组之间的区分准确度为血清分类为81.9-91.7％，尿分类为77.7％。我们的结果表明，PBC显示出独特的代谢组学指纹，这导致人们猜测能量代谢受损，可能与肠道菌群改变有关。PBC和CD显示了两个不同的代谢指纹。这些数据可为了解PBC的致病机理和检测新的治疗靶标提供线索。