John Vierling, MD, FACP, FAASLD, AGAF

Presentation and Diagnosis of PBC

Video content above is prompted by the following:

with PBC? What are the most and least common symptoms of the disease?

What is the typical path to a diagnosis for patients with PBC?

What clinical and laboratory assessments do you perform, and what markers do you look for?

Videos

Primary biliary cholangitis (PBC) often presents with fatigue and pruritus as the most common symptoms, while jaundice and xanthomas are less frequent. The diagnostic pathway typically involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key laboratory assessments include elevated alkaline phosphatase levels and the presence of antimitochondrial antibodies (AMA). Liver biopsy may be performed to confirm diagnosis and assess disease stage. Imaging, such as ultrasound or MRI, helps rule out other liver conditions. Early diagnosis is crucial for effective management and improved patient outcomes.

The panelists discuss the typical presentation of primary biliary cholangitis (PBC), covering common and uncommon symptoms, the diagnostic journey, and key clinical and laboratory assessments used to identify disease markers.

Pathophysiology of PBC

Could you explain the underlying pathophysiology of PBC and how it contributes to liver damage?

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the progressive destruction of small and medium-sized bile ducts within the liver. This destruction is primarily mediated by autoreactive T lymphocytes targeting the epithelial cells of the bile ducts. The ensuing cholestasis leads to the accumulation of toxic bile acids, causing hepatocellular damage and inflammation. Over time, this chronic inflammatory process results in fibrosis and eventually cirrhosis. The pathophysiology of PBC is influenced by genetic predispositions and environmental factors, contributing to its complex etiology and progression.

The panelists explain the pathophysiology of primary biliary cholangitis (PBC), highlighting how immune-mediated processes contribute to progressive liver damage in affected patients.

John Vierling, MD, FACP, FAASLD, AGAF

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