Primary Sclerosing Cholangitis: A Rare but Serious Liver Disease

Author : Raj Dhote | Published On : 04 Mar 2024

Primary Sclerosing Cholangitis (PSC) is a rare chronic liver disease characterized by inflammation and scarring of the bile ducts within the liver. As the bile ducts become damaged, they can start to narrow preventing bile from draining properly. Over time, this can lead to serious complications such as liver cirrhosis or liver failure requiring transplantation. In this article, we will take an in-depth look at what causes PSC, its symptoms, diagnosis and available treatment options.

What is Primary Sclerosing Cholangitis?

Primary Sclerosing Cholangitis (PSC) is an autoimmune disease where the immune system attacks the bile ducts of the liver causing them to become inflamed, scarred and narrowed over time. Bile is a fluid that is normally produced by the liver to help with fat digestion. It travels through the bile ducts to the small intestine. In PSC, the inflammatory process causes the bile ducts to develop strictures or blockages leading to a buildup of bile within the liver. This causes damage to the small bile ducts (cholangiopathy) and eventually the entire liver.

Causes and Risk Factors

While the exact cause of Primary Sclerosing Cholangitis remains unknown, it is considered an autoimmune disease. Genetics are thought to play a role as PSC often runs in families and is associated with certain genetic markers. Environmental triggers are also suspected to contribute such as infections or the gut microbiome. The strongest known risk factor is inflammatory bowel disease (IBD), most often ulcerative colitis which is present in 70-80% of PSC cases. Other potential risk factors include smoking, primary sclerosing cholangitis, liver flukes and infections such as salmonella. Men are affected 2-3 times more often than women. PSC usually develops in the 30-50 age group.

Symptoms

In the early stages, PSC may not cause any noticeable symptoms. As the disease progresses, common symptoms can include:

- Fatigue and weakness
- Itching without a rash (pruritus)
- Abdominal pain, discomfort or swelling in the upper right section
- Clay or pale colored stools
- Dark urine
- Loss of appetite and unintended weight loss
- Jaundice (yellowing of the skin and eyes)

These symptoms occur as the blocked bile ducts prevent normal bile flow, leading to a buildup of bile in the liver and bloodstream. Later stage symptoms indicate more advanced liver damage such as easy bruising, fluid retention or confusion.

Diagnosis

If a person presents with concerning symptoms, blood tests will show elevated liver enzymes and bilirubin levels suggestive of a liver problem. Imaging tests such as abdominal ultrasound, MRI or CT are then used to visualize the anatomy of the bile ducts. The gold standard test for diagnosing PSC is cholangiography which uses dye and imaging to directly visualize the bile ducts and detect any narrowing, dilations or strictures which are hallmarks of the disease. A liver biopsy may also be performed to examine cells under a microscope for signs of inflammation and scarring.

Treatment and Management

There is currently no cure for PSC but treatments aim to slow disease progression, relieve symptoms, improve liver function and prevent complications. Ursodeoxycholic acid (UDCA) has been shown to modestly improve liver biochemistry and may modestly slow disease progression when taken long term. It works by making bile less toxic to the bile ducts.

For those with additional ulcerative colitis, medications to control intestinal inflammation may help liver disease activity as well. Antibiotics are sometimes used for dominant strictures or infections in the bile ducts. Vitamin supplements may be recommended due to potential malabsorption issues. Liver transplantation is the only potential cure and considered for those with advanced scarring, frequent cholangitis or hepatocellular carcinoma. Lifelong follow-up with a hepatologist is important for ongoing monitoring and management.

Complications and Prognosis

Failure to control inflammation and scarring in PSC can lead to serious complications including liver cirrhosis in 20-30% of patients within 10 years of diagnosis. Cirrhosis increases the risk of further issues such as ascites (fluid buildup), esophageal/gastric varices, hepatic encephalopathy and liver cancer. Up to 15% of those with cirrhosis will require transplantation within 5 years. The 5 and 10-year transplant free survival rates are approximately 80-85% and 60-70% respectively; however, outcomes depend greatly on the stage and severity at diagnosis. With proper management, many are able to live long and healthy lives.

In summary, Primary Sclerosing Cholangitis is a chronic inflammatory condition affecting the bile ducts. While the causes are not fully known, early diagnosis and treatment can help reduce complications, manage symptoms and potentially slow disease progression in many cases. Further research into better treatments and a cure are ongoing to improve quality of life for those impacted by this rare disease.

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