Primary Biliary Cholangitis: Symptoms, Treatment


Primary biliary cholangitis, formerly called primary biliary cirrhosis, is a chronic disease in which the small ducts in the liver are slowly destroyed. When it happens, bile that aids with digestion and helps the body get rid of cholesterol, toxins and worn-out red blood cells will accumulate in the liver, leading to liver scarring, cirrhosis, and eventually liver failure.

Primary biliary cholangitis is believed to be an autoimmune disease. This means your healthy bile duct cells are mistakenly attacked by the immune system. Researchers reckon a combination of genetic and environmental factors plays a role in the development of primary biliary cholangitis.

It is estimated that in the United States, about 65 out of every 100,000 women and about 12 out of every 100,000 men have this disease.


About 6 in 10 people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. They may experience symptoms over the next 5 to 20 years. Comparatively, people who have symptoms at diagnosis typically have poor outcomes.

Common early symptoms involve:

  • Fatigue
  • Itchy skin
  • Dry eyes and mouth

Later signs and symptoms may include:

  • Pain in the upper right abdomen
  • Swelling of the spleen
  • Bone, muscle or joint pain
  • Swollen feet and ankles
  • Buildup of fluid in the abdomen due to liver failure
  • Fatty deposits on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees
  • Yellowing of the skin and eyes
  • Darkening of the skin that’s not related to sun exposure
  • Weak and brittle bones
  • High cholesterol
  • Diarrhea
  • Underactive thyroid
  • Weight loss
  • Dark urine


The exact cause of primary biliary cholangitis remains unclear, but it has an association with an overactive autoimmune system. In this disease, certain types of white blood cells called T cells start to collect in the liver and mistakenly destroy the healthy cells rather than detect and help defend against germs. Genetic and environmental factors trigger the condition. Possible environmental factors may include:

  • Infections
  • Cigarette smoking
  • Exposure to certain chemicals

Risk factors

  • Sex. Women are more likely to develop primary biliary cholangitis than men.
  • Age. People who are 30 to 60 years old have a high risk.
  • Genetic factors. If someone in your family has or had this disease, you will have a higher chance of getting it.
  • Geography. Primary biliary cholangitis is most common in northern Europe and North America.


If your liver damage keeps worsening because of primary biliary cholangitis, you may have complications such as:

  • Liver scarring (cirrhosis)
  • Increased pressure in the portal vein (portal hypertension)
  • Enlarged spleen (splenomegaly)
  • Gallstones and bile duct stones
  • Enlarged veins (varices)
  • Liver cancer
  • Weak bones (osteoporosis)
  • Vitamin deficiencies
  • Decreased mental function (hepatic encephalopathy)
  • Increased risk of other diseases


In addition to asking about your health history, your family’s health history, and performing a physical exam, your doctor may use the following tests to diagnose primary biliary cholangitis:

  • Blood tests
  • Cholesterol test
  • Liver tests
  • Antibody tests for signs of autoimmune disease

Although imaging tests is not necessary, they may be ordered to help confirm a diagnosis or rule out other conditions with similar signs and symptoms. These tests may include:

  • Ultrasound
  • Magnetic resonance cholangiopancreatography (MRCP)
  • Magnetic resonance elastography (MRE)
  • Endoscopic retrograde cholangiopancreatography (ERCP)

A liver biopsy may be needed if the diagnosis is still uncertain with the tests above. It can not only confirm the diagnosis but also determine the extent of the disease.


No cure is available for primary biliary cholangitis now. However, people with the condition can slow its progression and prevent complications with medications. These medications include:

  • Ursodeoxycholic acid (UDCA). This medication is also known as ursodiol (Actigall, Urso). It is commonly used at first. By helping move bile through the liver, UDCA improves liver function and reduce liver scarring.
  • Obeticholic acid (Ocaliva). Ocaliva is the newest medication for primary biliary cholangitis. According to studies, if given alone or combined with ursodiol for 12 months, people with the condition can improve liver function.
  • Fibrates (Tricor). Despite uncertainty about how it works, this medicine can reduce liver inflammation and itching in some people when they take it with UDCA.
  • Other medications. Other medications may be used including methotrexate (Trexall) and colchicine (Colcrys).

Furthermore, your doctor may recommend a liver transplant to prolong life if the condition can’t be kept under control with taking medications.

Lifestyle and home remedies

Here are some things you can do in your daily life to make you feel better:

  • Choose reduced-sodium foods
  • Never eat oysters ort other raw shellfish
  • Exercise most days of the week
  • Avoid alcohol

Check with your doctor before starting new medications or dietary supplements.

Keywords: primary biliary cholangitis; primary biliary cirrhosis.

Related Posts:

Cirrhosis: Symptoms, Causes, Treatment

Liver Transplant: Uses, Risk, Preparation

Liver Biopsy: Uses, Risk, Preparation

What Are the Different Stages of Liver Cirrhosis?

What Are the Diets for Cirrhosis Patients?

What is the Prognosis of Cirrhosis?

* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.