Primary biliary cholangitis (PBC), also referred to as primary biliary cirrhosis, is one of the most common immune-mediated liver diseases resulted from progressive destruction of the small bile ducts of the liver, causing bile and other toxins to accumulate in the liver, and also called cholestasis. PBC is characterized by the presence of circulating antimitochondrial antibodies (AMAs), bile duct pathology, progressive intrahepatic duct destruction, and cholestasis, and usually manifested by fatigue, itching, joint pain, and jaundice.
PBC is a relatively rare disease affecting all races and ethnicities throughout the world, but with a higher preponderance among women, especially middle-aged women over 40. As an autoimmune disorder, the pathogenetic mechanism of PBC is proposed to be associated with T cell lymphocyte-mediated dysregulated immune tolerance against mitochondrial antigen pyruvate dehydrogenase complex (PDC-E2) within biliary epithelial cells (BECs), which thereby results in biliary tract injury, progressive cholestasis, and liver fibrosis. The PBC can be diagnosed by several criteria: abnormal elevations in serum alkaline phosphatase (ALP); the detection of AMAs and related PBC-specific autoantibodies (e.g., anti-glycoprotein-210 antibody, anti-sp100 antibody, anti-p62 antibody); and nonsuppurative cholangitis affecting septal and interlobular bile ducts showed by the liver biopsy.
Fig.1 Model of the pathogenesis of PBC. (Louie, 2020)
Current management and treatment of patients with PBC aim to slow disease progression and control symptoms associated with chronic cholestasis, including frontline and second-line treatments. Multiple agents have been developed for the treatment of PBC.
Fig.2 Pathogenesis of PBC and targeted drug therapy. (Shah, 2020)
PBC is a rare autoimmune cholestatic liver disease. UDCA has been the standard and the only drug available for two decades, and a variety of novel agents and specific targeted therapies have been broken through in recent years. Adhering to the concept of striving for human health, Creative Biolabs has made great achievements in terms of new therapy development for PBC treatment. Based on the all-round technology platform and abundant experience, our professional scientist offers a series of solutions and services to facilitate successful PBC drug development.
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