PBC Basics

The signs and symptoms of primary biliary cholangitis (PBC) may not always correlate with the severity of the damage to the liver. While some patients may have severe symptoms and no severe damage in their liver, others may have severe liver damage with hardly any symptoms. However, research suggests that the earlier the symptoms appear, the faster the disease progresses.

Early signs and symptoms of PBC

Early signs and symptoms of PBC may include:

• Fatigue.
• Intense itching, known as pruritus.
• Pain in the joints or bones.
• Dry eyes and mouth.
• Dizziness.

The severity of these symptoms does not necessarily correlate with the severity of the liver disease. 

In the absence of any signs or symptoms, the diagnosis of PBC is usually done through a routine blood test, and most patients are diagnosed with PBC before they have any signs and symptoms.

One of the most common early signs of the disease, however, is pruritus (itchiness) seen in around 55% of patients, which usually affects the arms, legs and back. The exact cause of pruritus is not known, but it is thought to be caused by substances in the blood.

Signs and symptoms of advanced PBC

As the disease progresses and the damage to the liver increases, new symptoms may appear. These usually develop over five to 20 years from the time of diagnosis and may include:

• Jaundice or the yellowing of the skin and white of the eyes.
• Edema or the build-up of fluid in the lower legs and feet.
• Ascites or the build-up of fluid in the abdomen.
• Abdominal pain.
• Xanthelasma or cholesterol deposits on the skin, especially around the eyes, palms, soles, elbows or knees.
• Dark urine and pale, smelly stools.
• Hyperpigmentation in the skin.
• hypothyroidism or low levels of thyroid.

Symptoms of cirrhosis

In the very late stages of PBC, when cirrhosis develops, patients may develop the following additional symptoms. 

• Nausea.
• Weigh loss.
• Vomiting blood.
• Fever.
• Spider angiomas or spider-like blood vessels on the skin.

Complications associated with worsening liver damage

As the damage to the liver worsens, PBC can cause serious complications. These include:

• Portal hypertension or increased pressure in the portal vein, which carries blood to the liver, due to scarring.
• Varices may break and bleed, which can be life-threatening.
• Splenomegaly or the enlargement of the spleen due to the liver not being able to filter toxins out of the bloodstream properly.
• Gallstones and bile duct stones caused by unfiltered bile hardening and forming stones.
• Osteoporosis or weakened and brittle bones that may break easily.
• High cholesterol.
• Vitamin deficiencies, especially A, D, E and K vitamins, leading to night blindness and bleeding disorders.
• Hepatic encephalopathy or decreased mental function caused by the build-up of toxins.
• Increased risk of other diseases, such as Sjogren syndrome and those affecting the thyroid, skin and joints.
• Increased risk of liver cancer.

Reviewed by Harshi Dhingra, M.D., on December 18, 2023.