Primary Biliary Cirrhosis

Creator: Dr. Sanskriti Sakshi
Published: 2023-02-01T13:36:01

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Primary biliary cirrhosis is a condition in which inflammation and blockage of the bile ducts occur, impairing bile circulation. Read this article to know more.

Written by

Dr. Sanskriti Sakshi

Medically reviewed by

Dr. Ghulam Fareed

February 1, 2023

April 25, 2023

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Contents

What Is Primary Biliary Cirrhosis?
What Are the Reasons Responsible for Primary Biliary Cirrhosis?
What Are the Risk Factors of Primary Biliary Cirrhosis?
What Are the Signs and Symptoms of Primary Biliary Cirrhosis?
What Complications Do Primary Biliary Cholangitis Cause?
How Is Primary Biliary Cirrhosis Diagnosed?
How Is Primary Biliary Cirrhosis Treated?

Introduction

Primary biliary cirrhosis is also known by another name - primary biliary cholangitis (PBC). Primary biliary cholangitis is an autoimmune disease that causes swelling and inflammation of bile ducts. It is a chronic progressive liver disease that affects bile secretion. The incidence of PBC varies across the world. It is rare in Africa but common in northern Europe and North America. Although it affects both sexes, women are predominantly affected. It is also more commonly seen in smokers. The cause of PBC is not known clearly, as it is an autoimmune disease. Autoimmune disease refers to a condition in which the human body starts attacking its own healthy cells. Severe PBC cases cause liver fibrosis, eventually causing death due to liver failure. In addition, PBC is associated with high cardiac risk. Medicine can treat PBC. Liver transplantation is the last choice for PBC.

What Is Primary Biliary Cirrhosis?

Primary biliary cirrhosis or primary biliary cholangitis is a chronic progressive cholestatic liver disease. It is an autoimmune disease associated with the presence of antimitochondrial antibodies (AMA) in the body. PBC causes inflammation of the portal tracts. It is characterized by progressive damage to biliary ducts, eventually altering bile circulation. Bile is a yellowish-green fluid secreted by the liver. Bile is carried from the liver to the small intestine by the bile duct. Bile is neccesary for fat digestion and absorption. In PBC, bile secreted from the liver does not reach the small intestine due to damage to the bile ducts. The accumulation of bile in the biliary ducts causes inflammation and swelling of the bile ducts. As a result, liver fibrosis occurs, leading to liver cirrhosis.

What Are the Reasons Responsible for Primary Biliary Cirrhosis?

Primary biliary cirrhosis is caused due to genetic factors. The exact cause of PBC is unknown, but the immune mechanism is involved as it is an autoimmune disease. Genetic factors and environmental triggers are responsible for susceptible individuals. It is more common among cigarette smokers. The following are the causes of primary biliary cirrhosis:

Autoimmune non-hepatic disease such as thyroid disease (a condition that affects the thyroid gland).
Autoimmune hepatitis (liver inflammation).
Environmental trigger factors (tobacco).
Genetic factors.
Systemic sclerosis (an autoimmune disorder that affects the skin and internal organs).
Coeliac disease (a chronic immune disorder caused due to gluten ingestion).

What Are the Risk Factors of Primary Biliary Cirrhosis?

The following are the risk factors that increase the chances of developing PBC:

Smoking.
European citizens.
Women are more prone to PBC compared to men.
Previous history of genetic disorders in the family.
Urinary tract infections.
Older people (40 to 60 years) are more likely to be affected.

What Are the Signs and Symptoms of Primary Biliary Cirrhosis?

Primary biliary cirrhosis can present with insidious onset of pruritis or tiredness. Systemic symptoms are associated with PBC.

The following are the symptoms of PBC:

Fatigue.
Pruritis (itching).
Jaundice.
Upper abdomen discomfort.
Joint pain.
Fractures.
Weight loss.
Xanthamatous (a deposit of cholesterol) deposit generally found under the eyes.
Hepatomegaly (liver enlargement).
Splenomegaly (spleen enlargement).
Dryness of mouth or eyes.
Swelling in the limbs.
Hypothyroidism (a disease of the thyroid gland).
Loss of appetite.

What Complications Do Primary Biliary Cholangitis Cause?

Severe cases of PBC can cause liver fibrosis, leading to liver cirrhosis and death.

The following are the complication of PBC:

Liver Cirrhosis - Later stages of PBC causes liver cirrhosis. Cirrhosis is a liver disease resulting in impaired liver function.

Portal Hypertension - The abnormal increase in the pressure of portal venous circulation is called portal hypertension.

Splenomegaly - The enlargement in the size of the spleen is called splenomegaly.

Hepatomegaly - The enlargement of the size of the liver is called hepatomegaly.

Gallstones - The hardening and deposition of the digestive fluids result in the formation of gallstones. It is caused due to impairment of bile circulation.

Malabsorption - Prolonged impairment of bile circulation affects digestion resulting in malabsorption. In PBC, malabsorption of fat and vitamins occurs, which causes vitamin deficiency in the patient.

Osteoporosis - The bone density decreases, weakening bone strength and making it susceptible to fractures.

Jaundice - The condition in which skin, eyes, and nail yellowing occur due to high bilirubin deposition in the body.

Vitamin Deficiencies - Due to malabsorption, vitamin D and calcium deficiency occur in patients with PBC.

Liver Cancer - Carcinoma of the liver occurs at the end stages of fibrosis of the liver.

Liver Failure - Severe cases of PBC can cause liver dysfunction, eventually leading to liver failure.

Hypercholesterolemia - The accumulation of the excess amount of cholesterol and lipids in the body is called hypercholesterolemia.

Death - Lack of timely intervention can predispose affected person to further organ damages and serious ailments. The complications and damage caused by the impaired liver function can be fatal in individuals with prior disease conditions or otherwise.

How Is Primary Biliary Cirrhosis Diagnosed?

Primary biliary cirrhosis can be diagnosed incidentally by routine blood tests, as antimitochondrial antibodies are present in 95 % of the patients. PBC needs to be diagnosed in the early stage as later stages can develop portal hypertension and liver failure.

The following are the ways to diagnose PBC:

Blood Tests - A routine blood test indicates allergies if present. The elevated serum immunoglobulin levels give evidence of an autoimmune disease. Particularly IgM is found to be elevated in PBC.

Thyroid Test - The lower level of total thyroid stimulating hormones indicates hypothyroidism. Hypothyroidism should always be considered in patients with tiredness.

Lipid Profile Test - The elevated level of cholesterol confirms hypercholesterolemia. Hypercholesterolaemia is commonly found as the disease progresses.

Anti-bodies Test - Antimitochondrial antibodies (AMA) are found in 95 % of the patients. If AMA is absent, then anti-smooth muscle antibodies are found in 15 % of the patients. This test confirms the autoimmune disease.

Cholangiography - The histological evidence is obtained by cholangiography confirming biliary disease. It is the imaging technique for the bile ducts. The dye is injected into the patients intravenously, and the x-ray is taken at 10 minutes intervals to visualize the bile ducts.

Liver Function Tests (LFT) - The abnormal pattern of LFT shows evidence of cholestasis (a condition in which bile circulation gets impaired). In patients with PBC, the levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and gamma-glutamyl transferase (GGT) are elevated.

Liver Biopsy - A liver biopsy finds histological changes occurring in liver cells, if any, indicative of liver cirrhosis. A liver biopsy should be done in case of an uncertain diagnosis.

How Is Primary Biliary Cirrhosis Treated?

The first line of treatment for PBC is ursodeoxycholic acid (UDCA). Although, it is unclear whether drugs resolve all cases of PBC. Some patients who did not respond to UDCA require a second line of treatment. Symptomatic treatment regarding osteoporosis, malabsorption, and pruritis is suggested. Liver transplantation is the last choice in case of liver failure. The following are the treatment options for PBC:

1. Medication -

Ursodeoxycholic acid (UDCA) is the treatment of choice in PBC, as it improves bile flow. Clinically, UDCA has shown benefits in slowing down the progression of PBC, and it has few side effects.
Obeticholic acid (OCA) is a second-generation drug used in patients who are unresponsive to ursodeoxycholic acid.
Glucocorticoids, Azathioprine, and Ciclosporin, are immunosuppressant drugs used to suppress immune responses in case of autoimmune disease.
Cholestyramine can be given to treat pruritus.
Rifampicin and Naltrexone are used to eradicate infections.
Calcium and Vitamin D3, along with Bisphosphonates, are given to treat bone diseases (osteoporosis).

2. Dietary Supplements - Vitamin and protein supplements are suggested in case of malabsorption.

3. Liver Transplantation - A liver transplant should be considered in case of liver failure. Liver transplantation shows a survival rate of five years in affected individuals.

Conclusion

Primary biliary cirrhosis or primary biliary cholangitis is a chronic progressive cholestatic liver disease. It can cause severe complications if left untreated. The recovery in the early stages of primary biliary cirrhosis is good. Liver transplantation is the last choice in the later stages of the PBC., although there is a 30 % disease recurrence. The exact causes of PBC are unclear, but environmental factors trigger the condition in susceptible individuals. Quitting cigarette smoking is advisable as a preventive measure.

Frequently Asked Questions

1. What Is the Main Cause of Primary Biliary Cirrhosis?

It is unclear what causes primary biliary cirrhosis, also called primary biliary cholangitis. Several scientists believe it is an autoimmune disease where the body destroys its cells.

2. Is Primary Biliary Cirrhosis Curable?

No, it is not a curable disease. Even though there is no known cure for the disease currently, there are ways to treat the symptoms and slow its spread.

3. What Are the Four Stages of Primary Biliary Cirrhosis?

Primary biliary cirrhosis is a long-term illness that gets worse over time. These are the four stages of primary biliary cholangitis: Stage 1: inflammation and destruction of the bile ducts.
Stage 2: It is fibrosis or scarring of the liver tissue.
Stage 3: It is the stage of cirrhosis, or advanced scarring of the liver tissue, and
Stage 4: The stage of liver failure.
Primary biliary cholangitis can cause dry eyes and mouth, pain in the upper right side of the abdomen, spleen swelling, and muscle and joint pain.

4. Is Primary Biliary Cirrhosis Serious?

Primary biliary cirrhosis (PBC) is a serious disease that can cause severe liver damage and eventually lead to liver failure. PBC is a chronic disease that impairs the ability of the liver to function and can result in permanent scarring of liver tissue (cirrhosis).

5. How Long Can a Person Live With Primary Biliary Cirrhosis?

After being diagnosed, a person might not start having symptoms for two to four years, and some might not have any symptoms. People with PBC can live healthy lives without symptoms for at least 10 years after diagnosis.

6. What Is the Earliest Symptom of Primary Biliary Cirrhosis?

Most people with primary biliary cirrhosis do not feel sick in the early stages. But tiredness and skin that itches are often the first signs. Later, individuals might have pain in the upper right part of the stomach, dry eyes and mouth, a swollen spleen, and pain in the bones, muscles, or joints.

7. At What Age Does Biliary Cirrhosis Occur?

People between the ages of thirty and sixty are most likely to develop biliary cirrhosis.

8. What Is the New Treatment for Primary Biliary Cirrhosis?

Although there is no known cure for primary biliary cholangitis (PBC), medication can manage symptoms and slow the disease's progression. Ursodiol is a common treatment that aids in removing bile from the liver. People with PBC may sometimes require vitamin A, D, E, and K replacement therapy to replace the fat-soluble vitamins lost in fatty stools.

9. What Is the Difference Between Biliary Cirrhosis and Liver Cirrhosis?

Cirrhosis is scarring of the liver. Liver cirrhosis and biliary cirrhosis are both types of cirrhosis. But each one is caused by something different and affects another part of the liver. Liver cirrhosis is caused by long-term liver inflammation, usually caused by drinking too much alcohol or getting viral hepatitis. It affects the whole liver. When the bile ducts, which carry bile from the liver to the small intestine, become damaged, a condition known as biliary cirrhosis develops.

10. What Is the Best Treatment for Primary Biliary Cirrhosis?

Although there is no known cure for primary biliary cholangitis (PBC), medication can help manage symptoms and slow the disease's progression. Ursodiol is a medication that aids in the removal of bile from the liver. Doctors may also use medications, supplements, or changes to treat common PBC complications.

11. Can a Liver Fully Recover From Cirrhosis?

Cirrhosis is a serious disease that causes scarring and permanent liver damage. The liver can heal and regenerate itself over time, but only if it is still healthy and does not have a lot of scar tissue.

12. Can Primary Biliary Cirrhosis Cause Death?

Primary biliary cirrhosis (PBC) can be fatal. PBC can cause serious health problems as the liver damage worsens, including liver scarring (cirrhosis), which makes the liver work harder and may lead to liver failure. If PBC is not treated, Cirrhosis can lead to liver failure and even death.

13. What Does Primary Biliary Cirrhosis at the End Stage Mean?

Primary biliary cirrhosis, also called primary biliary cholangitis, is a long-term liver disease that makes the bile ducts inflamed and, in the end, permanently damaged. The disease can be in one of four stages based on how badly the liver is hurt. End-stage primary biliary cirrhosis is the last stage of the disease, which can cause the liver to stop working.

14. How Fast Does Primary Biliary Cirrhosis Progress?

Formerly known as primary biliary cirrhosis, primary biliary cholangitis is a chronic condition with a variable pace of progression. The onset of symptoms can lag by as much as ten to fifteen years after a diagnosis has been made.

15. What Can I Eat With Primary Biliary Cirrhosis?

Primary biliary cirrhosis patients should stick to a low-sodium, low-sugar, and low-fat diet. Fruits, vegetables, and lean meats should make up most of your diet. Getting all the nutrients needed from whole foods and supplements is crucial. Raw or undercooked seafood, pork, and milk may carry harmful germs and viruses and should be avoided to protect the liver. A personalized food plan can be developed with the help of a healthcare provider or a licensed dietitian.

Sources

Dr. Ghulam Fareed

Medical Gastroenterology

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