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Primary Biliary Cirrhosis

Dr Thng Yongxian

Hepatopancreatobiliary & General Surgeon
MBBS • MMed • MRCS (IRE) • FRCSED • FAMS

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What Is Biliary Cirrhosis?

Biliary cirrhosis is a chronic condition characterised by the progressive destruction of the bile ducts within the liver. This destruction hampers bile transport, a fluid essential for absorbing fats and fat-soluble vitamins in the small intestine.


The accumulation of bile leads to inflammation and scarring (fibrosis) in the liver, which can progress to cirrhosis, where normal liver tissue is replaced by scar tissue, severely impacting liver function.

Causes and Risk Factors

This condition is categorised into two main types: primary biliary cirrhosis (PBC) and secondary biliary cirrhosis (SBC). The causes and risk factors vary significantly between both types.

Primary Biliary Cirrhosis (PBC)

PBC is primarily considered an autoimmune condition, though its exact cause remains unclear. Key factors include:

  • Genetic predisposition: A family history of PBC or other autoimmune diseases can increase the risk.
  • Environmental triggers: Certain infections, smoking, and toxic substance exposure can be potential triggers for PBC.
  • Immune system dysfunction: The body’s immune system mistakenly attacks the cells lining the bile ducts, leading to inflammation and damage.

Secondary Biliary Cirrhosis (SBC)

SBC results from conditions that cause prolonged obstruction of the bile ducts, leading to bile accumulation and liver damage. Common causes include:

  • Bile duct obstructions: These can be due to gallstones, cysts, or tumours blocking the bile ducts.
  • Chronic biliary infection: Infections that affect the bile ducts can lead to scarring and obstruction.
  • Surgical complications: Procedures involving the bile ducts may inadvertently cause damage or strictures, leading to SBC.

Risk Factors

Apart from the direct causes, several risk factors increase the likelihood of developing biliary cirrhosis:

  • Age: PBC more commonly affects people between the ages of 30 and 60.
  • Gender: Women are more likely to develop PBC than men.
  • History of autoimmune diseases: Individuals with other autoimmune conditions, such as rheumatoid arthritis or thyroiditis, have a higher risk of PBC.

Symptoms

Biliary cirrhosis can progress asymptomatically, often presenting no symptoms until the condition is in an advanced stage. When symptoms do appear, they may include:

Early Symptoms

Fatigue: A pervasive sense of tiredness that is not relieved by rest

Itching (Pruritus): Unexplained, persistent itching, typically without a rash, affecting various body parts.

Dry eyes and mouth: Symptoms that are part of the sicca syndrome, which can be associated with autoimmune disorders.

As the Disease Progresses

  • Jaundice: Yellowing of the skin and whites of the eyes, resulting from the accumulation of bilirubin in the blood.
  • Dark urine and pale stools: Changes in urine and stool colour due to altered bilirubin processing.
  • Right upper quadrant pain: Discomfort or pain in the upper right part of the abdomen, near the liver.

Advanced Symptoms

  • Swelling in the legs and abdomen: Caused by fluid accumulation due to reduced protein production by the liver and increased pressure in the abdominal blood vessels.
  • Easy bruising and bleeding: As liver function declines, the production of proteins necessary for blood clotting decreases.
  • Bone weakness: Osteoporosis can develop, increasing the risk of fractures.

Diagnosis

Diagnosing biliary cirrhosis involves a combination of clinical evaluation, laboratory tests, and imaging studies to assess liver function and structure.

Clinical Evaluation

  • Medical history: To identify risk factors, symptoms, and any history of liver disease or autoimmune disorders.
  • Physical examination: Focuses on signs such as jaundice, skin changes, or liver enlargement.

Laboratory Tests

  • Blood tests: Measure liver enzymes, bilirubin levels, and specific markers of autoimmune activity against bile duct cells.
  • Immunoglobulin levels: Elevated IgM levels can be indicative of PBC.
  • Liver function tests: Assess the liver’s ability to produce proteins and process waste products.

Imaging Studies

  • Ultrasound: A method to visualise the liver and bile ducts, looking for signs of damage or obstruction.
  • Magnetic Resonance Imaging (MRI): Offer detailed images of the bile ducts and can identify blockages or structural changes.

Additional Tests

Additional diagnostic procedures may be recommended to rule out other conditions or confirm the diagnosis, such as endoscopic retrograde cholangiopancreatography (ERCP), which visualises the bile and pancreatic ducts more directly.

 

Understanding the available treatment options for biliary cirrhosis aids in managing the condition and improving patient outcomes.

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Non-Surgical Treatment Options

Non-surgical treatments focus on medication and lifestyle adjustments to manage symptoms and slow disease progression.

Medical Therapies

  • Ursodeoxycholic acid (UDCA): This bile acid is often the first line of treatment, aiming to slow disease progression by improving bile flow.
  • Obeticholic acid (OCA): For patients who do not respond adequately to UDCA, OCA offers an alternative to improve liver enzyme levels.
  • Immunosuppressive medications: These are used to manage autoimmune components of the disease, reducing inflammation and immune system activity.

Symptomatic Treatments

  • Cholestyramine: Specifically targets itching associated with bile accumulation.
  • Fat-soluble vitamin supplements: Supplements for vitamins D, E, A, and K to counteract deficiencies due to impaired bile flow.
  • Bone health medications: Treatments to prevent or manage osteoporosis, including calcium and vitamin D supplements.

Lifestyle Adjustments

  • Regular monitoring: Essential for adjusting treatment plans based on the progression of the disease.
  • Diet and exercise: A balanced diet and regular exercise support overall liver health.

Surgical Treatment And Other Procedures

Surgical treatments and procedures, including biliary drainage and liver transplantation, are considered for advanced cases or when non-surgical options are insufficient.

  • Biliary Drainage Procedures: For secondary biliary cirrhosis caused by obstruction, surgical intervention may be necessary to remove or bypass blockages.
  • Liver Transplantation: In cases where liver function is severely compromised, a liver transplant may be the most effective treatment, offering a chance for prolonged survival and improved quality of life.

Possible Complications

As the disease progresses, several complications may arise.

Portal hypertension

Increased blood pressure in the portal vein system could lead to varices and an increased risk of bleeding.

Liver failure

A severe reduction in liver function can lead to life-threatening complications.

Increased risk of liver cancer

Cirrhosis elevates the risk of developing liver cancer.

Liver failure

Reduced bone density, making bones more fragile and prone to fractures.

Dr Thng Yongxian

Senior Consultant

HEPATOPANCREATOBILIARY & GENERAL SURGEON

MBBS (SG) • MMed (Surg) • MRCS (Ire) • FRCSEd (Gen) • FAMS (Surg)

Dr Thng Yongxian is a dual fellowship-trained Hepatopancreatobiliary & General Surgeon who has performed over 6000 surgical procedures.

  • Bachelor of Medicine & Bachelor of Surgery
  • National University of Singapore
  • Membership of the Royal College of Surgeons, Ireland
  • Master of Medicine, General Surgery, Singapore
  • Fellow of the Royal College of Surgeons, Edinburgh
  • Fellow of the Academy of Medicine, Singapore

He pursued his subspecialty training in Hepatopancreatobiliary surgery first in the Department of Hepatopancreatobiliary Surgery, Singapore General Hospital. Following this, he completed a clinical fellowship in Minimally Invasive Hepatopancreatobiliary Surgery at Seoul National University Hospital in Bundang, South Korea.


Dr Thng set up and pioneered the Minimally invasive surgery program for his hospital in complex liver and pancreas cases. His surgeries were featured on live national television. His surgical videos have also been presented at international surgical conferences. He also pioneered the use of capsule colonoscopy for colorectal screening.


Dr Thng has served on various committees at hospital, cluster and ministry level. Dr Thng served as a member of the Ministry of Health (MOH) Laparoscopic cholecystectomy Clinician workgroup for value-driven care (VDC).

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Nexus Surgical @ Parkway East

Nexus Surgical @ Gleneagles Medical Centre

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Nexus Surgical @ Mt. Elizabeth Orchard

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Nexus Surgical @ Parkway East

Nexus Surgical @ Gleneagles Medical Centre

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