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Abstract：
UNASSIGNED: Primary biliary cholangitis (PBC) is a chronic cholangiopathy characterised by immuno-mediated injury of interlobular bile ducts leading to intrahepatic cholestasis and progressive liver fibrosis. PBC histology is characterised by portal inflammation, progressive fibrosis, ductopenia, and the appearance of the so-called ductular reaction. The aim of the present study was to investigate the pathogenetic relevance of ductular reaction in PBC.
UNASSIGNED: Liver biopsies were collected from naïve people with PBC (N = 87). Clinical-serological parameters were obtained at diagnosis and after 1 year of ursodeoxycholic acid (UDCA) treatment. Histological staging was performed on all slides according to multiple scoring systems and criteria for PBC. Liver samples were obtained from Mdr2 -/- mice treated with or without UDCA. Samples were processed for histology, immunohistochemistry, and immunofluorescence.
UNASSIGNED: Ductular reaction in people with PBC correlated with the disease stage and liver fibrosis, but not with disease activity; an extensive ductular reaction correlated with serum alkaline phosphatase levels at diagnosis, response to UDCA, and individuals\' estimated survival, independently from other histological parameters, including disease stage. In people with PBC, reactive ductules were associated with the establishment of junctions with bile canaliculi and with fibrogenetic cell activation. Consistently, in a mouse model of intrahepatic cholestasis, UDCA treatment was effective in reducing ductular reaction and fibrosis and increasing ductular-canalicular junctions.
UNASSIGNED: Extensive ductular reaction outlines a severe histologic phenotype in PBC and is associated with an inadequate therapy response and a worse estimated prognosis.
UNASSIGNED: In people affected by primary biliary cholangitis (PBC), the histological appearance of extensive ductular reaction identifies individuals at risk of progressive fibrosis. Ductular reaction at diagnosis correlates with the lack of response to first-line therapy with ursodeoxycholic acid and serves to restore ductular-canalicular junctions in people with PBC. Assessing ductular reaction extension at diagnosis may add valuable information for clinicians.
UNASSIGNED: Liver biopsies were collected from naïve people with PBC (N = 87). Clinical-serological parameters were obtained at diagnosis and after 1 year of ursodeoxycholic acid (UDCA) treatment. Histological staging was performed on all slides according to multiple scoring systems and criteria for PBC. Liver samples were obtained from Mdr2 -/- mice treated with or without UDCA. Samples were processed for histology, immunohistochemistry, and immunofluorescence.
UNASSIGNED: Ductular reaction in people with PBC correlated with the disease stage and liver fibrosis, but not with disease activity; an extensive ductular reaction correlated with serum alkaline phosphatase levels at diagnosis, response to UDCA, and individuals\' estimated survival, independently from other histological parameters, including disease stage. In people with PBC, reactive ductules were associated with the establishment of junctions with bile canaliculi and with fibrogenetic cell activation. Consistently, in a mouse model of intrahepatic cholestasis, UDCA treatment was effective in reducing ductular reaction and fibrosis and increasing ductular-canalicular junctions.
UNASSIGNED: Extensive ductular reaction outlines a severe histologic phenotype in PBC and is associated with an inadequate therapy response and a worse estimated prognosis.
UNASSIGNED: In people affected by primary biliary cholangitis (PBC), the histological appearance of extensive ductular reaction identifies individuals at risk of progressive fibrosis. Ductular reaction at diagnosis correlates with the lack of response to first-line therapy with ursodeoxycholic acid and serves to restore ductular-canalicular junctions in people with PBC. Assessing ductular reaction extension at diagnosis may add valuable information for clinicians.
摘要：
未经证实：原发性胆汁性胆管炎（PBC）是一种慢性胆管疾病，其特征是免疫介导的小叶间胆管损伤，导致肝内胆汁淤积和进行性肝纤维化。PBC组织学特征为门静脉炎症,进行性纤维化,导管减少症,以及所谓导管反应的出现。本研究的目的是研究PBC中导管反应的致病相关性。
未经证实：从患有PBC的幼稚患者（N=87）收集肝活检。在诊断时和熊去氧胆酸（UDCA）治疗1年后获得临床血清学参数。根据多个评分系统和PBC标准对所有载玻片进行组织学分期。从用或不用UDCA处理的Mdr2-/-小鼠获得肝脏样品。样本进行了组织学处理，免疫组织化学，和免疫荧光。
未经证实：PBC患者的导管反应与疾病分期和肝纤维化相关，但与疾病活动无关；诊断时广泛的导管反应与血清碱性磷酸酶水平相关，对UDCA的回应，和个人估计的存活率，独立于其他组织学参数，包括疾病阶段。在PBC的人中，反应性小导管与胆管连接的建立以及纤维细胞的激活有关。始终如一，在肝内胆汁淤积的小鼠模型中，UDCA治疗可有效减少导管反应和纤维化，并增加导管-小管连接。
UNASSIGNED：广泛的导管反应概述了PBC的严重组织学表型，并与不充分的治疗反应和较差的估计预后相关。
未经证实：在受原发性胆汁性胆管炎（PBC）影响的人群中，广泛导管反应的组织学表现表明个体有进行性纤维化的风险.诊断时的导管反应与对熊去氧胆酸一线治疗缺乏反应相关，并有助于恢复PBC患者的导管-小管连接。在诊断时评估导管反应扩展可能会为临床医生增加有价值的信息。
未经证实：从患有PBC的幼稚患者（N=87）收集肝活检。
未经证实：PBC患者的导管反应与疾病分期和肝纤维化相关，
UNASSIGNED：广泛的导管反应概述了PBC的严重组织学表型，并与不充分的治疗反应和较差的估计预后相关。
未经证实：在受原发性胆汁性胆管炎（PBC）影响的人群中，
