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Complications
By Mayo Clinic staffAs liver damage progresses, people with primary biliary cirrhosis may develop a number of serious problems, including:
- Cirrhosis. The term "primary biliary cirrhosis" isn't entirely accurate because cirrhosis develops only in the later stages of the disease — often many years after diagnosis. Yet when it does occur, cirrhosis can be life-threatening because it interferes with your liver's ability to carry out essential functions. Cases of primary biliary cirrhosis are divided into four stages. The first stage — inflammation of the bile ducts — is the least serious, and stage 4 — cirrhosis — the most serious. Ongoing cirrhosis can lead to liver failure, which occurs when your liver is no longer able to function.
- Increased pressure in the portal vein (portal hypertension). Blood from your intestine, spleen and pancreas enters your liver through a large blood vessel called the portal vein. When scar tissue blocks normal circulation through your liver, blood backs up, much like water behind a dam, leading to increased pressure within the vein. And because blood doesn't flow normally through your liver, hormones, drugs and other toxins aren't filtered properly before entering your bloodstream.
- Enlarged veins (varices). When circulation through the portal vein is slowed or blocked, blood may back up into other veins — mainly those in your stomach and esophagus. The blood vessels are thin walled, and increased pressure in your veins can cause bleeding in your upper stomach or esophagus. This bleeding is a life-threatening emergency that requires immediate medical care.
- Liver cancer. The destruction of healthy liver tissue that occurs in cirrhosis increases your risk of liver cancer.
- Weak bones (osteoporosis). Liver scarring interferes with your liver's ability to process vitamin D and calcium, both of which are essential for bone growth and health. As a result, weak, brittle bones and bone loss may be complications of late-stage primary biliary cirrhosis, and your doctor may order a bone density test to look for osteoporosis.
- Vitamin deficiencies. A lack of bile affects the absorption of fats and of the fat-soluble vitamins, A, D, E and K. This sometimes leads to deficiencies of these vitamins in advanced cases of primary biliary cirrhosis.
- Cognitive impairment. Some people with primary biliary cirrhosis have problems with memory and concentration. Cognitive difficulties don't seem to correlate directly to the amount of liver damage, however.
Other complications
In addition to bile duct and liver damage, people with primary biliary cirrhosis are likely to have other metabolic or immune system disorders, including:
- Thyroid disease. Thyroid problems are common in people with primary biliary cirrhosis. They may appear long before bile duct damage is diagnosed, or thyroid disorders may develop after you've received a diagnosis of primary biliary cirrhosis.
- Limited scleroderma (CREST syndrome). This immune system disorder is a subset of scleroderma, a disease that leads to thickening, tightening and hardening of connective tissue. CREST syndrome can affect many body systems, including your blood vessels and esophagus, and sometimes your digestive tract, lungs and heart. People with primary biliary cirrhosis generally have some, rather than all, of the signs and symptoms of CREST.
- Raynaud's phenomenon. One of the components of CREST, Raynaud's phenomenon may also occur in people with primary biliary cirrhosis. It occurs when small blood vessels (capillaries) spasm in response to cold or emotional stress, blocking the flow of blood. The areas of affected skin generally turn white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and may throb or tingle.
- Rheumatoid arthritis. Some people with primary biliary cirrhosis have the aching joints that typify rheumatoid arthritis, another autoimmune disorder.
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.
