Primary Biliary Cholangitis
Primary biliary cholangitis is a rare disease in which the bile ducts become progressively inflamed and destroyed. Bile builds up in the liver (cholestasis), causing fibrosis and cirrhosis of the liver.
Primary biliary cholangitis is somehow different from secondary biliary cholangitis, in which the bile ducts are obstructed or damaged due to another cause like a tumor.
Causes of Primary Biliary Cholangitis
The cause of primary biliary cholangitis is not fully understood but is considered as an autoimmune disease, in which the immune system attacks the body’s bile duct cells.
Some experts believe that primary biliary cholangitis can be triggered by a fungal, bacterial, or parasitic infection.
Women are 9 times more likely than men to develop primary biliary cholangitis.
Serious complications of primary biliary cholangitis include:
- Cirrhosis of the liver
- Portal hypertension
- Varices (enlarged veins in the esophagus and stomach that are susceptible to life-threatening bleeding)
- Liver cancer
Symptoms of primary biliary cholangitis
Some patients experience none of the symptoms, while others may experience symptoms like:
- Itchy skin
- Abdominal pain
- Dry eyes
- Dry mouth
- Yellowing of the skin and eyes (jaundice)
- Darkening of the skin
- Swollen feet and ankles
- Accumulation of abdominal fluid (ascites)
- Greasy diarrhea
- Memory problems
- Vitamin deficiencies
Treatments for Primary Biliary Cholangitis
There is no cure for primary biliary cholangitis, so treatments are aimed at slowing the progression of disease, relieving symptoms, and preventing complications.
Medications are used to reduce cholestasis, improve liver function, relieve itching and fatigue, and to treat complications like varices and osteoporosis. The first line of treatment is Ursodeoxycholic acid (UDCA), also known as ursodiol (Actigall, Urso). Medication and surgery for portal hypertension may also be needed.
If medical therapies no longer control symptoms and the liver begins to fail, liver transplantation may be considered. Primary biliary cholangitis can recur in the new liver after some years.