Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis (PSC)

Primary Sclerosing Cholangitis (PSC) is a chronic liver disease that affects the bile ducts, which are responsible for carrying bile from the liver to the small intestine. In PSC, the bile ducts become inflamed, scarred, and eventually damaged, leading to a reduction in bile flow. This can result in liver damage, cirrhosis, and eventually liver failure. At Digestive System Healthcare, we specialize in diagnosing and managing PSC to help patients maintain liver function and improve their quality of life.

 

What Causes Primary Sclerosing Cholangitis?

The exact cause of PSC is unclear, but it is believed to be an autoimmune disorder in which the body’s immune system attacks the bile ducts. Several factors may contribute to the development of PSC:

  • Genetics:
    • A family history of PSC or related autoimmune diseases may increase the risk.
  • Autoimmune response:
    • The immune system mistakenly targets the bile ducts, causing inflammation and scarring.
  • Other conditions:
    • PSC is strongly associated with inflammatory bowel diseases, especially ulcerative colitis.
  • Environmental factors:
    • While not fully understood, infections or toxins might play a role in triggering PSC in susceptible individuals.

What are the Symptoms of Primary Sclerosing Cholangitis?

Symptoms of PSC can be gradual and may vary in severity. Many individuals may remain asymptomatic in the early stages, but as the disease progresses, the following symptoms may appear:

  • Fatigue:
    • Persistent tiredness or exhaustion that doesn’t improve with rest.
  • Jaundice:
    • Yellowing of the skin and eyes, indicating liver dysfunction.
  • Itching (pruritus):
    • Itchy skin that can be severe, especially at night.
  • Abdominal pain:
    • Discomfort or pain in the upper right side of the abdomen.
  • Dark urine and pale stools:
    • Changes in stool color and urine due to bile obstruction.
  • Fever and chills:
    • Signs of a potential infection or inflammation.
  • Weight loss and loss of appetite:
    • Associated with liver dysfunction and bile flow issues.

How is Primary Sclerosing Cholangitis Diagnosed?

PSC is diagnosed through a combination of clinical evaluation and diagnostic tests, including:

  1. Blood tests:
    • To assess liver function, check for elevated liver enzymes, and detect markers of inflammation.
  2. Imaging tests:
    • Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) to visualize bile ducts and identify strictures or blockages.
  3. Liver biopsy:
    • A liver biopsy may be performed to assess the extent of liver damage and rule out other conditions.
  4. Ultrasound or CT scan:
    • To assess the liver and bile ducts for signs of damage or complications.

What are the Treatment Options for Primary Sclerosing Cholangitis?

Currently, there is no cure for PSC, but treatment focuses on managing symptoms, slowing the disease’s progression, and preventing complications. Treatment options include:

  • Ursodeoxycholic acid (UDCA):
    • Often prescribed to help improve bile flow and reduce liver inflammation, though its effectiveness may vary.
  • Endoscopic procedures:
    • To treat bile duct strictures or blockages, often using balloon dilation or stenting.
  • Antibiotics:
    • To treat infections in the bile ducts, which are common in PSC.
  • Liver transplantation:
    • In cases of advanced liver disease or liver failure, a liver transplant may be necessary.
  • Symptom management:
    • Medications to relieve itching, pain, and other symptoms associated with liver dysfunction.
  • Regular monitoring:
    • Routine liver function tests and imaging to monitor disease progression and detect complications early.