What You'll Learn Here:
- 1 Primary biliary cirrhosis signs and symptoms
- 2 Early-stage symptoms:
- 3 Later-stage symptoms:
- 4 Primary biliary cirrhosis causes and risk factors
- 5 Primary biliary cirrhosis complications
- 6 Diagnosing primary biliary cirrhosis
- 7 Treating primary biliary cirrhosis
- 8 Helpful primary biliary cirrhosis lifestyle changes
- 9 Prognosis and life expectancy
~ by Jo Jordan
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
- Dry eyes and mouth
- Intense itching, especially of the arms, back, and legs
- 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
- 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
- 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)
- 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
- 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.
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 Primary biliary cirrhosis (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