Research advances in animal models of primary biliary cholangitis / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is an inflammatory and cholestatic liver disease caused by autoimmune response targeting the small- and medium-sized intrahepatic bile ducts. The most specific manifestations of this diseases in clinical practice were positive anti-mitochondrial antibody and selective destruction of small- and medium-sized bile ducts based on liver histology. Since it is difficult to obtain the clinical samples of early-stage PBC, the construction and optimization of mouse models is an important method to investigate the pathogenesis of PBC. An understanding of the modeling principles of PBC animal models and disease features in serology, histology, and cytology not only helps to improve the awareness of PBC, but also helps to design scientific and rational research protocols.

Research advances in animal models of primary biliary cholangitis / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is an inflammatory and cholestatic liver disease caused by autoimmune response targeting the small- and medium-sized intrahepatic bile ducts. The most specific manifestations of this diseases in clinical practice were positive anti-mitochondrial antibody and selective destruction of small- and medium-sized bile ducts based on liver histology. Since it is difficult to obtain the clinical samples of early-stage PBC, the construction and optimization of mouse models is an important method to investigate the pathogenesis of PBC. An understanding of the modeling principles of PBC animal models and disease features in serology, histology, and cytology not only helps to improve the awareness of PBC, but also helps to design scientific and rational research protocols.

Clinical characteristics of primary biliary cholangitis with thyroid disease / 中华风湿病学杂志

Objective:To investigate the clinical features of primary biliary cholangitis (PBC) with thyroid disease (TD) and the association between TD and PBC.Methods:From 2005 to 2017, clinical data of PBC patients from the affiliated hospital of Qingdao university were retrospectively analyzed. All PBC patients were divided into 2 groups according to whether they have TD. The general conditionsand clinical manife-stations in the two groups were analyzed. T-test, nonparametric test, Chi-square test and Fisher's exact test-swere applied to compare datain subgroups. Results:A total of 148 PBC patients were involved in to our study, of which 45 cases (30.4%) had TD. PBC patients with TD showed a higher incidence of Sj?gren's synd- rome (SS) (33.3% vs 17.5%, χ2=4.545, P=0.033). Moreover, there was a higher positive rate of anti-SP100 and anti-SSB antibody in PBC patients with TD (20.0% vs 5.8%, χ2=5.440, P=0.020; 20.0% vs 2.9%, χ2=10.087, P=0.001) compared with patients without. PBC patients without TD presented a higher incidence of abdominal distension and jaundice (29.1% vs 11.1%, χ2=5.629, P=0.018; 23.3% vs 8.9%, χ2=4.241, P=0.039) compared to patients with TD. The ratio of patients with elevated total bilirubin (TBiL), direct bilirubin (DBiL), or increased alkaline phosphatase (ALP) was higher in PBC without TD group(40.8% vs 17.8%, χ2=7.405, P=0.007; 43.7% vs 17.8%, χ2=9.147, P=0.002; 69.9% vs 51.1%, χ2=4.811, P=0.028). Correspondingly, PBC patients without TD was associated with a higher probability of cirrhosis and portal hypertension (40.8% vs 22.2%, χ2=4.731, P=0.030; 25.2% vs 8.9%, χ2=5.183, P=0.023). Conclusion:TD has no effect on the natural history of PBC. PBC patients with TD are associated with a lower probability of liver fibrosis, portal hypertension and cholestasis symptoms but a higher incidence of SS when compared with PBC patients without TD. Multi-disciplinary approach should be implemented to the mag-nagement of PBC.

Immunological pathogenesis of primary biliary cholangitis / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune disease caused by the attack of the liver by the immune system and is commonly seen in middle-aged women.The pathological features of this disease include the infiltration of a large number of innate immune cells (NK cells,NK T cells,and monocytes,etc.) and adaptive immune cells (T lymphocytes and B lymphocytes) at the portal area and injury of small intrahepatic bile ducts.In addition,PBC patients have high serum levels of anti-mitochondrial antibodies and inflammatory cytokines such as interferon-γ,tumor necrosis factor-α,interleukin-6,and interleukin-12.Although current studies have shown that autoreactive T cells are the major effector cells for PBC,NK cells,NK T cells,monocytes,and B cells around the portal vein also directly or indirectly participate in the development and progression of PBC.This article systematically summarizes the role of innate immune cells,adaptive immune cells,and related chemokines in the pathogenesis of PBC,in order to provide a theoretical basis for a deep understanding of the immunological pathogenesis of PBC.

Application prospects of research on pathogenesis of primary biliary cirrhosis / 临床肝胆病杂志

Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by the destruction of small intrahepatic bile ducts. Incomprehensible and complicated autoreactive responses participate in the development and progression of PBC, which involve various immune cells and inflammatory mediators. Based on the aspects of innate immunity and adaptive immunity, this article summarizes recent advances in the research on PBC pathogenesis at cellular and molecular levels and evaluates the clinical application of these studies. This article not only gives a feasible direction for researchers and clinicians in this study field, but also provides a theoretical basis for clinical diagnosis and novel therapeutic strategies.