A chronic autoimmune liver disease known as primary biliary cirrhosis (PBC) is defined by the progressive loss of the intrahepatic bile ducts, which results in cholestasis, inflammation, and liver parenchyma fibrosis. It is thought to be caused by a confluence of immunological dysregulation, environmental variables, and genetic predisposition, while the precise etiology is yet unknown.The presence of antimitochondrial antibodies (AMA) in the serum of afflicted patients is a defining characteristic of PBC and helps in the diagnosis of the illness. These antibodies specifically target the bile duct epithelial cells' mitochondria, setting off an immune-mediated onslaught that ultimately results in their demise. This impairs bile flow, which in turn causes bile acids and other harmful compounds to build up in the liver. This eventually causes the liver to deteriorate and scar.PBC often manifests clinically as exhaustion, hepatomegaly (enlarged liver), pruritus (itching), and abdominal pain in the right upper quadrant. Patients may experience complications such liver failure, portal hypertension, and cirrhosis as the condition progresses. Further depressing the quality of life are extrahepatic symptoms such xanthelasmas, osteoporosis, and sicca syndrome (dry mouth and eyes).A combination of imaging scans, laboratory testing, clinical characteristics, and liver biopsies are used to diagnose PBC. Common laboratory findings in PBC include elevated levels of gamma-glutamyl transferase (GGT) and alkaline phosphatase (ALP), as well as the presence of AMA. Imaging techniques that can determine the degree of liver damage and rule out alternative causes of liver illness include transient elastography (FibroScan), magnetic resonance cholangiopancreatography (MRCP), and ultrasound.The goals of PBC management are to reduce symptoms, stop the disease from getting worse, and avoid consequences. The first-line treatment is ursodeoxycholic acid (UDCA), which has been demonstrated to enhance liver biochemistry and postpone the need for liver transplantation in certain individuals. Patients who do not respond well to UDCA may be evaluated for other pharmaceutical treatments, such as obeticholic acid (OCA). The only effective treatment for end-stage PBC that can also be a cure is liver transplantation.In summary, intrahepatic bile duct damage causes cholestasis, inflammation, and liver fibrosis in Primary Biliary Cirrhosis, a chronic autoimmune liver disease. Improving results and the quality of life for those who are impacted requires early diagnosis and effective treatment.
