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Primary Biliary Cirrhosis

What Is Primary Biliary Cirrhosis (PBC)?

~ by Jo Jordan

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Primary biliary cirrhosis (PBC) is a chronic disease of the liver that results in the destruction of the liver’s bile ducts.

Made in the liver, bile is a digestive liquid that travels through the bile ducts to the gallbladder, and then into the small intestine where it helps digest fats and fat-soluble vitamins. When the bile ducts become damaged by chronic inflammation – eventually disappearing altogether – bile accumulates in the liver.

Gradually the liver cells are damaged causing cirrhosis or scarring (scar tissue, fibrosis); the liver loses its ability to function, and life-threatening complications develop.

Primary Biliary Cirrhosis Signs and Symptoms

Because PBC advances slowly, many patients lead active lives, and experience no symptoms up to ten to fifteen years, sometimes longer, following diagnosis.

But as scar tissue replaces healthy liver tissue, the flow of blood through the liver becomes partly blocked, interfering with the organ’s ability to perform many of its key functions, such as

Controlling infections
Making the proteins that regulate blood clotting
Processing drugs, hormones, and nutrients
Producing bile to help absorb fats
Removing bacteria and toxins from the blood
Replacing its own damaged cells, effectively

Early-stage symptoms:

Dry eyes and mouth
Fatigue
Intense itching, especially of the arms, back, and legs

Later-stage symptoms:

Digestive problems (diarrhea and/or steatorrhea: greasy, frothy, and foul-smelling stools)
Fatty deposits in the skin around the eye (xanthomas)
Fluid build-up in abdomen, ankles, and feet
Jaundice
Skin darkening
Urinary tract infections

Primary Biliary Cirrhosis Causes and Risk Factors

While the exact cause of PBC is unknown, some researchers suggest it may be an autoimmune condition. This kind of disorder leads the immune system – which normally protects the body – to attack the body’s own tissues, in this case the bile ducts.

But the autoimmune disorder theory has some flaws. PBC rarely occurs in children, even though autoimmune conditions typically begin early in life. Nor does PBC usually respond to drugs that suppress the immune system, the way most autoimmune disorders do.

Scientists, therefore, suspect other factors – such as exposure to certain chemicals; bacterial (i.e. urinary tract infections), fungal, or parasitic infections; and genetics – play a role in triggering the onset of PBC:

Genetics – while not technically hereditary (i.e. a disease caused by a specific gene or genetic defect passed from parent to child) PBC tends to run in families
Gender – over ninety percent of PBC sufferers are women¹
Age – most people diagnosed with PBC are between forty and sixty years of age²

Primary Biliary Cirrhosis Complications

Many complications can develop once PBC progresses to cirrhosis:

Ascites (abnormal accumulation of fluid in the abdomen)
Bruising and bleeding
Cirrhosis
CREST syndrome (an immune system disorder related to scleroderma, CREST leads to thickening, tightening, and hardening of connective tissue)
Edema (the swelling of soft tissues as a result of excess water accumulation)
Enlarged veins
Esophageal varices (abnormally enlarged veins in the lower esophagus and upper part of the stomach)
Gallstones
Gastropathy (stomach disease)
Hepatic encephalopathy (brain and nervous system damage resulting from liver damage)
Immune system dysfunction (leading to the risk of infection)
Insulin resistance and type 2 diabetes
Kidney failure
Liver cancer
Lung failure
Maldigestion
Osteoporosis (weak bones)
Portal hypertension (increased pressure in portal vein)
Raynaud's phenomenon (small blood vessel spasm in response to cold or emotional stress, in which blood flow becomes blocked)
Renal stones
Rheumatoid arthritis
Sensitivity to medications
Splenomegaly (enlargement of the spleen)
Thyroid disease
Vitamin deficiencies

Diagnosing Primary Biliary Cirrhosis

Because the disease progresses slowly and many patients experience no symptoms, the presence of PBC often turns up as a result of abnormal results on routine liver function blood tests.

Various tests are necessary to confirm a PBC diagnosis:

Abdominal ultrasound
Anti-mitochondrial antibody (AMA) blood test (ninety percent of PBC sufferers have this antibody)³
Cholesterol blood test
Liver function tests – blood testing for an elevated level of alkaline phosphatase (a liver enzyme)
Liver biopsy
Magnetic resonance elastography (MRE) – a relatively new test, MRE combines magnetic resonance imaging (MRI) with low-frequency sound waves to help reveal physical properties of tissues and organs: i.e. liver stiffness of may indicate cirrhosis

Treating Primary Biliary Cirrhosis

There is no cure for PBC, and no known treatment for reversing its progression. Treating the disease in its early stages may slow liver damage and the onset of cirrhosis; early treatment aims to relieve symptoms and prevent complications.

Improving liver function:

For maintaining healthy liver functioning throughout life, liver cleansings are sometimes recommended. Working to improve digestion, a liver cleanse can be an aid to overall health.

Research indicates that when prescribed early, ursodiol/Actigall (also known as ursodeoxycholic acid, UDCA) – a naturally occurring bile acid – can improve liver function, retard liver failure and the necessity for liver transplantation during end-stage liver disease, and extend overall life expectancy.⁴

Ursodiol – which rarely causes side effects – must be taken daily for life, or until liver transplantation.

Relieving itching:

Minor skin itching can sometimes be relieved through regular colon cleansings.

Calcium supplements and vitamin replacement therapy are sometimes recommended to relieve itching, as well as the following medications:

Cholestyramine and colestipol – cholesterol-lowering drugs may provide relief, but can be extremely constipating; they also interfere with the metabolizing of ursodiol, hormones, some vitamins, and other medications
Liver transplant – sometimes the only option for intolerable itching
Opioid antagonists – must be administered intravenously
Rifampin – it’s slow acting, and can cause other side effects or toxic reactions

Reducing fluid retention:

Diuretic medication helps remove excess fluid from the body. If bacterial peritonitis is present in excess abdominal fluid, oral antibiotics may be prescribed to prevent infection.

Controlling portal hypertension:

A beta-blocker or nitrate may be prescribed to lower the pressure in enlarged veins, and reduce the risk of bleeding.

Treating hepatic encephalopathy:

Bowel cleansing and sometimes the administration of antibiotics may be indicated, as well as a reduction of dietary protein. For maintaining healthy colon and bowel functioning throughout life, colon cleansings are sometimes recommended. Lactulose – a laxative given orally or via enema – is another bowel cleansing treatment.

Treating hepatorenal syndrome (kidney failure):

Hemodialysis treatment is required, which is the removal of waste from the blood via machine. Medications may also be required to improve blood flow through the kidneys.

Helpful Primary Biliary Cirrhosis Lifestyle Changes

Many PBC patients feel better when they implement a healthy lifestyle. Doing so can relieve and may even prevent some of the symptoms:

Avoid alcohol consumption
Do not eat raw shellfish; it may contain infection-causing bacterium
Drink a sufficient amount of water
Eat nutritiously
Ensure adequate exercise
Practice good skin care to reduce itching; take cool baking soda or oatmeal baths, and avoid irritating clothes, soaps, and detergents
Reduce stress
Regular colon and/or liver cleansing
See your dentist regularly
Take calcium and vitamin D supplements
Use artificial tears for dry eyes

Prognosis and Life Expectancy

While normally slow, the progression of PBC varies considerably. For some people, symptoms do not appear for a dozen years or more after onset; others become seriously ill within three to five years. Once symptoms develop, however, life expectancy is approximately ten years.⁵

PBC is in an advanced stage when itching disappears, the fatty deposits under the skin shrink, and jaundice develops. Twenty-five percent of PBC cases progress to end-stage liver disease.⁶

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Notes:
1. Mayo Foundation for Medical Education and Research (MFMER), “Primary biliary cirrhosis,” http://www.mayoclinic.com/health/primary-biliary-cirrhosis/DS00604 (accessed February 16, 2009).
2. National Digestive Diseases Information (NDDIC), a service of the National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, “Primary Biliary Cirrhosis,” http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/ (accessed February 16, 2009).
3. Ibid.
4. American Liver Foundation, “Primary Biliary Cirrhosis (PBC),” http://www.liverfoundation.org/education/info/pbc/ (accessed February 16, 2009).
5. The Merck Manuals Online Medical Library, “Primary Biliary Cirrhosis,” http://www.merck.com/mmhe/sec10/ch136/ch136d.html (accessed February 16, 2009).
6. J. Neuberger, “Primary biliary cirrhosis,” Medicine, Volume 35, Issue 2, Pages 79-82, http://linkinghub.elsevier.com/retrieve/pii/S1357303906007031 (accessed February 16, 2009).