Primary biliary cirrhosis (or PBC) is a condition in which the bile ducts (ducts that remove bile from the liver) become irritated and inflamed, eventually causing damage to the liver cells and cirrhosis (irreversible scarring). Most patients with PBC are adult women, usually between 35 and 60 years of age.
Unfortunately, more than half of those with PBC have no symptoms when they are diagnosed. Doctors become aware of the disease after analyzing routine blood work for other conditions or reasons. The symptoms are often very vague; they may include abdominal pain or fatigue. As the condition progresses, symptoms like jaundice (yellowing of skin or eyes), fatty stools, itching and fatty deposits under the skin or eyelids occur.
Diagnosis and Treatment
Anyone who believes they may have PBC or have had abnormal blood tests should make an appointment with a hepatologist for further examination and a definitive diagnosis.
Laboratory testing for routine liver function is usually the first step in diagnosis. Other blood tests that are more specific for PBC are also performed. A liver ultrasound examination can be performed to determine the size of the bile ducts, and a biopsy may be needed to confirm the diagnosis.
Medication may be prescribed to reduce the itching and to address other symptoms. Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement may be added to prevent or treat soft, weakened bones (osteomalacia). In some cases, a liver transplant may also be an option.
As a member of the NewYork-Presbyterian Healthcare System, hepatologists and NYPBMH are participants in the Liver Transplant Program. Our team of hepatologists, nutritionists, nursing and support staff works with the patient throughout the entire process.