Primary Biliary Cholangitis

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What is Primary biliary cholangitis (PBC)?

Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease in which the body’s immune system mistakenly attacks the small bile ducts inside the liver. Over time, this leads to bile buildup, inflammation, and gradual destruction of liver tissue, resulting in fibrosis (scarring) and potentially cirrhosis if untreated.

Bile is essential for digesting fats and removing toxins. When bile cannot flow properly (a condition called cholestasis), it damages liver cells and causes systemic symptoms. PBC primarily affects middle-aged women, but men and younger adults can develop it as well.

Is Primary biliary cholangitis common?

PBC is considered uncommon but not rare. It affects an estimated 1 in 1,000 women over the age of 40. Early diagnosis and modern treatments have significantly improved outcomes, helping most patients maintain normal life expectancy with proper care.

Can Primary biliary cholangitis be cured?

PBC cannot be cured, but its progression can be slowed or controlled with medication and lifestyle changes. When detected early, most patients can manage symptoms and prevent liver failure. In advanced stages, liver transplantation may be required.

Causes

What causes Primary biliary cholangitis?

The exact cause of PBC is unknown, but it is believed to involve a combination of genetic and environmental factors that trigger the immune system to attack the liver’s bile ducts.

Common contributing factors include:

  • Autoimmune reaction – the immune system targets bile duct cells
  • Genetic predisposition – family history of PBC or other autoimmune diseases
  • Environmental triggers – infections, smoking, or exposure to certain chemicals
  • Hormonal influences – more common in women, suggesting a role of estrogen

PBC often coexists with other autoimmune diseases such as thyroid disease, Sjögren’s syndrome, or rheumatoid arthritis.

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*Price Effective 12/1/2025
$125
Initial Visit
$95
Follow Up

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Symptoms

What are the symptoms of Primary biliary cholangitis?

Symptoms may develop gradually and often go unnoticed in early stages. Common signs include:

  • Persistent fatigue (most common early symptom)
  • Itchy skin (pruritus), often worse at night
  • Dry eyes and mouth
  • Jaundice (yellowing of skin and eyes) in later stages
  • Dark urine and pale stools
  • Upper right abdominal discomfort
  • Swelling in the legs or abdomen (due to fluid buildup)
  • Bone pain or fractures from weakened bones (osteoporosis)

Diagnosis

How is Primary biliary cholangitis diagnosed?

PBC is usually detected through routine blood tests before symptoms appear. Diagnosis involves:

  • Liver function tests (LFTs) – showing elevated alkaline phosphatase (ALP) levels
  • Antimitochondrial antibody (AMA) test – present in about 90–95% of PBC patients
  • Liver biopsy – to confirm diagnosis and assess stage of liver damage
  • Imaging studies – ultrasound, CT, or MRI to rule out bile duct obstruction
  • FibroScan® – non-invasive test to assess liver stiffness and fibrosis

Treatment

How is Primary biliary cholangitis treated?

While there’s no cure, treatments can slow disease progression, reduce symptoms, and prevent complications.

Medications:

  • Ursodeoxycholic acid (UDCA) – improves bile flow and liver function; first-line treatment
  • Obeticholic acid (OCA) – used when UDCA alone isn’t effective
  • Fibrates – may help lower liver enzymes and control itching in some patients
  • Antihistamines or bile acid binders – to relieve itching
  • Vitamin supplements – for fat-soluble vitamin deficiencies (A, D, E, K)

Lifestyle and supportive measures:

  • Avoid alcohol and hepatotoxic drugs
  • Maintain a healthy diet and weight
  • Manage fatigue with rest and gentle exercise
  • Protect bone health through calcium and vitamin D

Procedures:

  • Liver transplantation – considered for end-stage liver disease or severe complications; outcomes are generally excellent

References

Medically reviewed by:

Dr. Javeed Siddiqui, MD, MPH

Dr. Siddiqui is the Chief Medical Officer at TeleMed2U responsible for clinical and technical program development as well as maintaining a thriving telemedicine practice in infectious diseases which includes specialized care of Hepatitis and HIV.

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Affordable – with or without insurance

With Insurance

Hepatology

Your copay
Depending on insurance

Without Insurance

*Price Effective 12/1/2025
$125
Initial Visit
$95
Follow Up

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