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What is Primary Biliary Cholangitis

Primary Biliary Cholangitis (PBC), formerly known as Primary Biliary Cirrhosis, is a chronic autoimmune disease that progressively damages the bile ducts in the liver. Bile ducts are small channels responsible for carrying bile, a fluid that helps with digestion and the elimination of waste products from the body. When these ducts become inflamed or damaged in PBC, bile can accumulate in the liver, leading to liver damage and potentially to cirrhosis or liver failure over time.
Key Features of PBC:
Cause: The exact cause is unknown, but PBC is believed to be an autoimmune condition where the immune system mistakenly attacks the bile ducts.

Genetic predisposition and environmental factors (like infections or exposure to toxins) may contribute.

Diagnosis: Blood tests that can help diagnose primary biliary cholangitis (PBC) include: 

  • Anti-mitochondrial antibody (AMA): Found in the blood of about 95% of people with PBC. 
     
  • Alkaline phosphatase (ALP): Higher-than-normal levels of this liver enzyme indicate a problem with bile flow through the liver. 
     
  • Gamma-glutamyl transferase (GGT): Higher-than-normal levels of this liver enzyme indicate a problem with bile flow through the liver. 
     
  • Enhanced Liver Fibrosis (ELF) test: Measures three chemicals in the blood that indicate liver scarring. 
     
  • Anti-kelch-like 12 (KLHL12) and anti-hexokinase 1 (HK1): Autoantibodies that are highly specific for PBC. 
     
Other blood tests that may be used include:
  • Total bilirubin
  • Aminotransferase levels (ALT, AST)
  • Albumin
  • Prothrombin time
  • Cholesterol and triglyceride levels
  • Antinuclear antibodies (ANAs) such as anti-sp100 and antiglycoprotein 210 (anti-gp210) antibodies 
    Other tests that may be used to diagnose PBC include: Imaging tests such as x-rays and ultrasounds, Liver biopsy, Magnetic resonance cholangiopancreatography (MRCP), and Magnetic resonance elastography (MRE).

Symptoms: Early stages are often asymptomatic (found incidentally through abnormal liver tests).

Common symptoms include fatigue, itching (pruritus), dry eyes/mouth, and upper abdominal discomfort.

Advanced disease may lead to jaundice, dark urine, pale stools, and signs of cirrhosis.

Risk Factors:

Risk Factors: More common in women (about 90% of cases).

Typically diagnosed between ages 30 and 60.

Family history and certain environmental triggers may increase risk.

Treatments:  Ursodeoxycholic acid (UDCA): A bile acid that helps improve bile flow and slows disease progression.

Two new treatments for Primary Biliary Cholangitis (PBC) have recently received FDA approval, offering additional options for patients who do not respond adequately to first-line therapies like ursodeoxycholic acid (UDCA):

  1. Elafibranor (Iqirvo): Approved in June 2024, this medication targets patients with insufficient response to UDCA or those unable to tolerate it. It works by reducing liver enzyme levels such as alkaline phosphatase (ALP) without exacerbating itching, a common symptom of PBC. It can be used as monotherapy or in combination with UDCA. Clinical trials demonstrated its efficacy in improving liver biomarkers while minimizing side effects.
  2. Seladelpar (Livdelzi): Approved in August 2024, this therapy is similarly aimed at patients not adequately managed by UDCA. Livdelzi has shown promise in not only improving liver enzymes and reducing bilirubin levels but also alleviating symptoms such as itching and fatigue. This drug also offers flexibility in being used alone or alongside UDCA.

Both drugs represent advancements in personalized treatment approaches for PBC, addressing the unmet needs of patients who do not achieve optimal results with existing options like UDCA or Ocaliva (obeticholic acid). Confirmatory trials for these medications are ongoing to further evaluate their long-term efficacy and safety

Symptom Management: Antihistamines or cholestyramine for itching. Supplements for vitamin deficiencies if malabsorption occurs. Advanced cases may require a liver transplant.

If you or someone you know has been diagnosed with or is experiencing symptoms of PBC, it’s essential to work closely with a healthcare provider to manage the disease and monitor liver health.