Understanding Primary Sclerosing Cholangitis (PSC): Symptoms, Causes, Diagnosis, and Treatment

Key Takeaways

  • This progressive autoimmune disease causes inflammation and scarring of bile ducts both inside and outside the liver, with 75% of patients also having ulcerative colitis as a co-existing condition.
  • Alkaline phosphatase levels are typically elevated 3-10 times normal values, while MRCP (magnetic resonance cholangiopancreatography) shows characteristic "string of beads" appearance in bile ducts for diagnosis.
  • Men are affected twice as often as women, with most cases diagnosed between ages 30-50, and patients have a 10-20% lifetime risk of developing bile duct cancer (cholangiocarcinoma).
  • No medications can halt disease progression, but ursodeoxycholic acid may improve liver function tests, and cholestyramine at 4-8 grams daily helps control severe itching.
  • Liver transplantation has excellent outcomes with 85% five-year survival rates, and may be necessary within 10-15 years of diagnosis in many patients due to progressive liver failure.

Primary sclerosing cholangitis (PSC) is a disease that affects your bile ducts, which are tubes that carry bile from your liver to your small intestine. Bile is a digestive fluid that helps break down fat from food. In people with PSC, the bile ducts become inflamed and develop scar tissue, which can block the flow of bile. This blockage can cause bile to build up in the liver, leading to liver damage and other complications. PSC is more common in males.

Symptoms of PSC

PSC often develops slowly, and you may have the disease for years before noticing any symptoms. Some common signs and symptoms of PSC include:

  • Fatigue

  • Itchy skin

  • Yellowing of the skin and eyes (jaundice)

  • Fever and chills (if bile ducts become infected)

  • Right upper quadrant abdominal pain

  • Dark Urine

  • Pale Stools

Causes of PSC

The exact cause of PSC is not known, but it may be related to factors such as:

  • Viral or bacterial infections

  • Genetic factors

  • Problems with the immune system

PSC is also linked to inflammatory bowel disease, particularly ulcerative colitis and, less commonly, Crohn's disease. About 75% of people with PSC also have ulcerative colitis, although the connection between these conditions is not fully understood.

Understanding Primary Sclerosing Cholangitis (PSC): Symptoms, Causes, Diagnosis, and Treatment

Diagnosing PSC

To diagnose PSC, your doctor may start by ordering a blood test to check your liver enzyme levels. Abnormal levels of certain enzymes can indicate the presence of PSC. To confirm the diagnosis, your doctor may recommend one of the following imaging tests:

  • Magnetic resonance cholangiopancreatography (MRCP): This non-invasive test uses magnets and radio waves to create detailed images of your bile ducts.

  • Endoscopic retrograde cholangiopancreatography (ERCP): During this test, a long, flexible tube called an endoscope is inserted through your mouth and into your digestive tract. Dye is then injected into your bile ducts, and X-rays are taken to visualize any blockages or abnormalities.

  • Percutaneous transhepatic cholangiography (PTC): This test involves inserting a needle through your skin and into a bile duct in your liver. Dye is injected through the needle, and X-rays are taken to examine your bile ducts.

Treatment Options for PSC

Currently, there are no medications specifically approved for treating PSC. However, your doctor may recommend the following to help manage symptoms and complications:

  • Medications to relieve itching

  • Antibiotics to treat infections in the bile ducts

  • Vitamin supplements to address any deficiencies

  • Endoscopic procedures to open blocked bile ducts (during ERCP)

In cases of advanced liver disease or liver failure, a liver transplant may be necessary. Most people with PSC who undergo a liver transplant experience good outcomes and improved quality of life.

If you suspect you may have PSC or are experiencing any of the symptoms mentioned above, it's essential to consult with your healthcare provider for an accurate diagnosis and appropriate treatment plan. Researchers are continually working on developing new therapies for PSC, and your doctor may be able to provide information about clinical trials testing promising medications.

For more information about primary sclerosing cholangitis, visit the following reputable sources:

The Bottom Line

Early detection through blood tests and imaging is crucial since this disease progresses silently toward liver failure over 10-20 years. Regular monitoring for complications like bile duct cancer and preparing for potential transplantation are essential parts of management. If you're experiencing persistent fatigue, itching, or jaundice, Doctronic can help evaluate your symptoms quickly.

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