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Symptoms
By Mayo Clinic staff
Early stage
Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience a number of symptoms early in the disease:
- Fatigue. A common symptom of primary biliary cirrhosis is fatigue, but doctors haven't found any correlation between the degree of exhaustion and the severity of the illness. This means that people with mild primary biliary cirrhosis and those with more serious disease may be equally fatigued.
- Itching. Another common symptom, itching (pruritus), is often most bothersome over your legs, arms and back. The severity of itching may change, often becoming worse at night and improving during the day. Nighttime itching can disturb sleep, making fatigue worse and sometimes leading to depression. The cause of this severe itching isn't clear.
- Dry eyes and mouth (sicca syndrome). Sicca syndrome often occurs in people with other autoimmune disorders. It causes inflammation in the moisture-secreting glands of the eyes and mouth, resulting in the decreased production of tears and saliva.
Later stage
As the destruction of bile duct and liver cells progresses, other signs and symptoms may develop, such as:
- Jaundice. A common sign of advanced liver disease, jaundice turns your skin and the whites of your eyes yellow. The discoloration is due to high blood levels of bilirubin, a byproduct of the breakdown of the hemoglobin from old or damaged red blood cells. Normally, bile carries bilirubin out of your liver so that it can be excreted from your body. But as more bile ducts are destroyed and the flow of bile slows, bilirubin begins to build up in your blood and eventually jaundice becomes visible in your skin and eyes.
- Hyperpigmentation. Inadequate bile flow increases the production of the skin pigment melanin. This causes your skin to become darker, even in areas that aren't exposed to the sun. Sometimes the deeper color isn't uniform, and your skin appears blotchy.
- Swollen feet (edema) and abdomen (ascites). As liver damage progresses, your body begins to retain salt and fluids. At first, the excess salt and water accumulate mainly in your feet and ankles (edema), which tend to become more swollen late in the day. In time, fluid can also collect in your abdomen.
- Cholesterol deposits (xanthomas). Your body uses bile as the main way of eliminating excess cholesterol. When disease interferes with this process, the amount of cholesterol in the blood increases. This can lead to the formation of fatty deposits in the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees. These raised, waxy growths usually don't appear until blood cholesterol reaches very high levels. Even then, not everyone with primary biliary cirrhosis develops them.
- Digestive problems. Because bile is essential for the digestion and absorption of fats, primary biliary cirrhosis can cause intestinal problems. These include diarrhea and steatorrhea — greasy, bad-smelling stools that result from poor fat digestion.
References
- Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.htm. Accessed Aug. 14, 2009.
- Primary biliary cirrhosis. American Liver Foundation. http://www.liverfoundation.org/education/info/pbc/. Accessed Aug. 14, 2009.
- Cirrhosis. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/sec03/ch026/ch026c.html. Accessed Aug. 14, 2009.
- Friedman LS. Liver, biliary tract and pancreas disorders. In: McPhee SJ, et al. Current Medical Diagnosis & Treatment. 48th ed. Los Altos, Calif.: Lange Medical Publications; 2009. http://www.accessmedicine.com/content.aspx?aID=7993. Accessed Aug. 14, 2009.
- Kaplan M, et al. Primary biliary cirrhosis. New England Journal of Medicine. 2005;353:1261.
- Kaplan MM. Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Kaplan MM. Overview of the treatment of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Hirschfeld GM, et al. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. New England Journal of Medicine. 2009;360:2544.
- Taouli B, et al. Advanced MRI methods for assessment of chronic liver disease. American Journal of Roentgenology. 2009;193:14.
- Lazaridis K, et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. Hepatology. 2007;46:785.
- Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. New England Journal of Medicine. 2007;357:1254.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Aug. 31, 2009.
- Hay JE. Bone disease in cholestatic liver disease. Gastroenterology. 1995;108:278.
