| Summary: | Primary biliary cholangitis (PBC) is characterized by the impaired enterohepatic circulation of bile acids caused by immune-mediated destruction of the intrahepatic bile ducts, leading to progressive liver dysfunction. This report describes the case of a patient with PBC accompanied with diabetes mellitus who exhibited a remarkable improvement in glycemic control after treatment with ursodeoxycholic acid (UDCA) and bezafibrate. A 51-year-old woman initially referred our department with poorly controlled diabetes during the previous 2 years. The patient had failed to achieve a favorable glycated hemoglobin (HbA1c) level (8.7%) despite treatment with oral glucose-lowering agents, and abnormal liver biochemistry findings indicated predominant increases in serum alkaline phosphatase and γ-glutamyltranspeptidase levels. The patient was diagnosed with PBC based on the presence of cholestatic liver profile and positive tests for anti-mitochondrial antibody, and treatment with UDCA and bezafibrate was then initiated. After a 5-week treatment with these agents, all liver biochemistries and hallmarks of inflammation showed immediate improvement. Notably, her HbA1c level was remarkably ameliorated to 6.3% within 5 weeks after the initiation of treatment, and adequate glycemic control (HbA1c level < 6.5%) was maintained without any additional glucose-lowering agents for the succeeding 3 years. Metabolic profiles revealed that the glucose-lowering effect of the treatment was due to improved pancreatic β cell function and insulin sensitivity possibly via incretin effects and eliminating hepatic inflammation. This case may be interesting when considering the relationship between bile acids and glucose metabolism, and may offer an option for the treatment of glucose intolerance accompanied with cholestatic liver diseases. Keywords: Primary biliary cholangitis, Diabetes mellitus, Bile acids, Hepatic inflammation, Ursodeoxycholic acid, Bezafibrate
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