Primary biliary cirrhosis, a disease of the bile ducts, has been around for a long time. More recently, there has been a movement to change the name to primary biliary cholangitis. The presence of the word cirrhosis in the name is both misleading, and a cause of concern for patient’s that have it. Not all patients with PBC have cirrhosis. Cholangitis, which is inflammation of the bile ducts, seems to be a more representative term to use. At Liver Specialists of Texas, we have extensive experience in treating those patients with PBC.
PBC is considered an autoimmune disease, that affects the bile ducts of the liver. It is progressive in nature, and can lead to further destruction of liver tissue and the eventual development of cirrhosis. Generally, it is a slowly progressive disease, and is in most cases, progression is judged in decades rather than years. In your liver, bile ducts are the extensive system of tubes draining bile from your liver to your gallbladder and intestines. With PBC you develop damage to the bile ducts, the bile has a tendency to get trapped in the liver, causing damage. What causes PBC is really not fully understood at this time. It is not contagious, nor is it caused by alcohol. Most of us feel that primary biliary cholangitis is inherited, as we see it occasionally cluster and family’s.
Top Five Facts
- Primary biliary cholangitis typically affects young white female.
- To make the diagnosis of PBC, the lab test for the antimitochondrial antibody is positive and the majority of cases, about 95%.
- Fatigue is the most common symptom of PBC, no nonspecific, occurs in approximately 78% of the cases.
- UDCA is the only FDA approved medication for the treatment of primary biliary cholangitis.
- Patients with primary biliary cholangitis can present with a number of systemic complaints and complications throughout the body including:
- Pruritus ( itching) occurring in 20-70% of patients
- Elevated cholesterol
- Vitamin D deficiency
- Osteoporosis in approximately 30% of individuals
- Sicca syndrome (dry eyes and/or mouth)