Primary Biliary Cirrhosis

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.

  Related Topics

  ---

  Sickle Cell Hepatopathy- Symptoms and Treatment

  Jaundice in Adults: An Overview

  Laparoscopic Liver Biopsy - Types, Indications, and Procedure

• 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.