PDF(Pubmed)
Abstract：
UNASSIGNED: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur in IBD with the use or even in the absence of thiopurine treatment. We aimed to study the significance of the presence of NRH and obliterative portal venopathy (OPV), both causes of non-cirrhotic portal hypertension (NCPH), in patients having PSC.
UNASSIGNED: Patients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003-2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records.
UNASSIGNED: Thirty-one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn\'s disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic-appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use.
UNASSIGNED: The current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy.
UNASSIGNED: Patients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003-2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records.
UNASSIGNED: Thirty-one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn\'s disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic-appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use.
UNASSIGNED: The current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy.
摘要：
未经证实：原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，其特征是肝内和肝外胆管的炎症和纤维化。PSC通常与炎症性肠病(IBD)相关。结节性再生增生（NRH）可在IBD中使用或甚至在不使用硫嘌呤治疗的情况下发生。我们的目的是研究NRH和闭塞性门静脉病（OPV）的存在的意义，非肝硬化门脉高压（NCPH）的两种原因，在患有PSC的患者中。
UNASSIGNED：从涵盖2003-2019年期间的数字病理学数据库中确定了PSC和同时进行肝活检的NRH患者。肝活检的评估和原始诊断在基于NRH和OPV诊断的标准组织学特征的审查中得到证实。从电子病历中获得临床和实验室数据。
未经证实：31名患者（21名男性，10名女性；活检时的中位年龄40.1岁）被纳入研究。12例（38.7％）患者在肝活检中除NRH外还进行了OPV。19例(61.2%)患者患有IBD，其中11例患有克罗恩病(CD)，7与溃疡性结肠炎（UC），1例不确定结肠炎。13例(41.9%)患者有门静脉高压的证据,10例（32.2%）食管静脉曲张，4例（12.9%）有静脉曲张破裂出血史，6（19.3%）伴腹水，脾肿大14例（12.9%）。11例（35.4％）患者在影像学上出现肝硬化。12例（38.7％）患者有既往或目前使用硫嘌呤的病史。
未经证实：目前的研究表明，有或没有OPV的NRH独立发生在PSC患者中，并可能导致NCPH，即使在没有并发IBD和/或噻嘌呤治疗的情况下。
UNASSIGNED：从涵盖2003-2019年期间的数字病理学数据库中确定了PSC和同时进行肝活检的NRH患者。肝活检的评估和原始诊断在基于NRH和OPV诊断的标准组织学特征的审查中得到证实。从电子病历中获得临床和实验室数据。
未经证实：31名患者（21名男性，10名女性；活检时的中位年龄40.1岁）被纳入研究。12例（38.7％）患者在肝活检中除NRH外还进行了OPV。19例(61.2%)患者患有IBD，其中11例患有克罗恩病(CD)，7与溃疡性结肠炎（UC），1例不确定结肠炎。13例(41.9%)患者有门静脉高压的证据,10例（32.2%）食管静脉曲张，4例（12.9%）有静脉曲张破裂出血史，6（19.3%）伴腹水，脾肿大14例（12.9%）。11例（35.4％）患者在影像学上出现肝硬化。12例（38.7％）患者有既往或目前使用硫嘌呤的病史。
未经证实：目前的研究表明，有或没有OPV的NRH独立发生在PSC患者中，并可能导致NCPH，即使在没有并发IBD和/或噻嘌呤治疗的情况下。
