Primary Biliary Cirrhosis (PBC): Signs, Causes, Diagnosis And Treatment Of This Autoimmune Disease Of Liver

What Is Primary Biliary Cholangitis/Cirrhosis? Hepatologist and other various experts share everything about this liver disease.

Tavishi Dogra
Written by: Tavishi DograPublished at: Feb 05, 2021
Primary Biliary Cirrhosis (PBC): Signs, Causes, Diagnosis And Treatment Of This Autoimmune Disease Of Liver

What is Primary biliary cirrhosis? Primary biliary cirrhosis is an autoimmune condition in which the body's immunity starts working against oneself. Usually, the immunity is against the infections, but in Primary biliary cirrhosis, the immunity begins working against the body which is more common in females than in the males and is associated with progressive liver damage. There are four stages, and if it goes unabated, it usually leads to extensive fibrosis to the liver, which will lead to the end-stage liver disease, thereby causing cirrhosis. This disease is also associated with other autoimmune disorders like dermatological problems, Sjogren's syndrome, diseases related to the eyes and the bones etc. What are the possible causes of primary biliary cirrhosis (PBC)? It is caused due to the activation of the autoimmune process and sometimes happens due to viral illness or without any apparent known cause. It may be associated with certain foodstuff, bacterial infections, or genetically inherited diseases where the autoimmune process gets activated at a certain age due to certain precipitating factors.

What are the signs of primary biliary cirrhosis (PBC)?

signs of primary biliary cirrhosis

Dr Neerav Goyal (Senior Consultant and Head, Liver Transplant, at Indraprastha Apollo Hospitals) states that "patients will usually have itching all over the body, and they might develop jaundice and yellowing of the eyes, dark colour urine, and the stool colour may become pale. As the disease progresses, they may have a recurring fever, cholangitis. They may have joint, bone pains as arthritis may occur, dry eyes, dry tongues. The main presentation is deranged Liver tests and Liver Failure. As it goes, the patients may develop fatigue weight loss, fluid in the abdomen, episode of blood vomiting due to enlarged veins, bleeding through the rectum and swelling in the legs."

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What is the diagnosis of primary biliary cirrhosis (PBC)?

"Diagnosis is made by blood tests (high Alkaline phosphate level), auto autoimmune antibodies (Anti mitochondrial antibodies), and deranged Liver Function tests. Primary biliary cirrhosis is detected on specific blood tests and in the case of autoimmune diseases mostly anti mitochondria antibody (AMA) which is elevated in almost 90 -95% of the patients, is detected on liver biopsy Post the diagnosis is confirmed, patients are started on certain drugs to decrease the liver enzyme-like deoxycholic sodium acid. In case patients are suffering from symptoms like for itching, they are put on steroids to control the inflammatory process" says Dr Neerav Goyal. 

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What symptoms indicate a complication of primary biliary cirrhosis?

Most primary biliary cirrhosis complications are related to cirrhosis and start after primary biliary cirrhosis progresses to cirrhosis. In some cases, portal hypertension and may develop before cirrhosis.

 complication of primary biliary cirrhosis

  • Portal hypertension: This condition is one of the common complications of cirrhosis. Portal hypertension may lead to other complications, such as:
  • Oedema—swelling due to a buildup of fluid—in the ankles, feet, or ascites and legs—a buildup of fluid in the abdomen
  • Varices also called as enlarged blood vessels, in the oesophagus, stomach, or both
  • An enlarged spleen, called splenomegaly
  • Splenomegaly: Splenomegaly may cause the spleen to enlarge and retain platelets and white blood cells, reducing these platelets and cells' numbers in the blood. 

What is the expected duration of Primary biliary cirrhosis? Explains Dr Neerav Goyal

People with primary biliary cirrhosis may not develop any signs or symptoms for up to ten years. And if a person has the first stage of primary biliary cirrhosis (PBC stage 1 or 2), their life expectancy is average. If a person with PBC has advanced signs and symptoms as seen in an advanced stage, the average life expectancy is about ten to fifteen years.

Also Read: Ayurveda and Liver Health: Ayurvedic Herbs and Remedies For Liver Problems

Primary biliary cirrhosis: When should one seek medical advice?

Primary biliary cirrhosis is chronic and progressive. It is not curable, and over time can lead to permanent liver damage. It is advisable to seek medical advice in the early stage, i.e. in stage 01. Primary biliary cirrhosis usually develops slowly. Many people generally live for many years without any symptoms. And once they develop signs, proper medication can help manage them.

Primary biliary cirrhosis

Who is at risk of developing primary biliary cirrhosis? 

Dr Goyal shares that, "Primary biliary cirrhosis is found commonly in women rather than men. There may be an ancestral element to the growth of primary biliary cirrhosis (PBC) since a person with the condition is likely to have a family member also afflicted. Women are 9 times more likely than men to develop (PBC) primary biliary cirrhosis. It most often occurs in the middle age between the ages of forty to sixty."

Also Read: How Are Hepatitis and Cirrhosis Different?

What are the treatment and prevention options available for primary biliary cirrhosis? 

Yes, it is possible to prevent primary biliary cholangitis. Prevention can be possible by doing the following: intake of calcium supplements and regular exercise. "There are few medications available for its treatment. Most important is to control itching in these patients. (UDCA) Ursodeoxycholic Acid is the most common and first line of treatment for these patients. Very recently, a new drug called Obeticholic Acid has been launched in India for treatment of PBC. Despite treatment, some PBC patients progress to Liver Failure. For these patients, Liver Transplant is very useful and lifesaving. To conclude, PBC is a rare but treatable disease" says Dr Gaurav Gupta, Sr. Consultant & Chief Surgeon - Liver Transplant & HPB Surgery, Fortis Hospital, Mulund.

Also Read: Suffering from Liver Cirrhosis? Here Are Some Effective Ayurvedic Tips To Tackle The Condition

 treatment and prevention options

Here are some FAQs related to Primary Biliary Cirrhosis answered by Dr Akash Shukla, Senior Hepatologist, Global Hospital, Mumbai

  • What is the total number of primary biliary cirrhosis cases? The following are the real primary biliary cirrhosis cases seen worldwide every year: Rare between 10 - 50 patients.
  • What is the general age group for primary biliary cirrhosis cases? Most primary biliary cholangitis occurs in the following age groups: Aged between 45-65 years.
  • Some essential facts of primary biliary cirrhosis (PBC): Those patients with PBC who show a better response to ursodeoxycholic acid may come across the general public than life expectancy. PBC affects nine women per man. Do you know that many PBC sufferers never have cirrhosis?
  • What if primary biliary cirrhosis untreated? Primary biliary cirrhosis causes complications if it is not treated. The list below is of problems that may arise BY leaving untreated primary biliary cirrhosis: spleen enlargement, cirrhosis, osteoporosis, hepatic encephalopathy, intravenous expansion.
  • Self-care tips for primary biliary cirrhosis: The following self-care tasks or lifestyle changes may help in the treatment or management of primary biliary cholangitis:
  • Choosing a low sodium diet: Helps prevent tissue inflammation and creates fluid in the abdominal cavity
  • Avoid eating oysters or other raw shellfish: Helps in the prevention of severe liver diseases
  • Regular exercise: Reduces the risk of bone loss and increases bone density
  • Avoid alcohol consumption: Helps reduce the risk of liver damage.

Conclusion

Dr Gaurav Gupta concludes that Primary Biliary Cholangitis (PBC) previously known as Primary Biliary Cirrhosis (PBC), is a sporadic Liver disease. Incidence of this disease worldwide is 0.3-5.8 in 100,000 individuals every year. It is more commonly present in middle-aged females as compared to males (10:1). This is an autoimmune disease with a genetic predisposition. This disease involves the destruction of small bile ducts in the Liver. It leads to Cholestasis (a condition in which the Liver does not secrete bile acid)."

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