Primary Sclerosing Cholangitis (PSC)
What is it?
Primary sclerosing cholangitis (PSC) is a progressive disease of the liver and gallbladder characterized by inflammation of the bile ducts. This inflammation cause scarring of the bile ducts which Eventually, can lead to cirrhosis of the liver and liver failure and can increases the risk of various cancers. The itiology of PSC is unknown. Genetics, immune system dysfunction, and abnormal composition of the gut flora may play a role. PSC is a rare disease and most commonly affects people with IBD
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Primary Sclerosing Cholangitis (PSC).
Signs & symptoms
Although Nearly half of people with PSC do not have symptoms, Signs and symptoms of PSC may include:
Severe itching
Fatigue
Jaundice
Enlargement of the liver and spleen
Abdominal pain
Diagnosis
PSC is diagnosed based on having at least two of three clinical criteria after secondary causes of sclerosing cholangitis have been ruled out:
- Serum alkaline phosphatase (ALP) large than 1.5x the upper limit of normal for longer than 6 months
- Cholangiography demonstrating biliary strictures or irregularity consistent with PSC
- Liver biopsy consistent with PSC (if available)
A farther imaging tests can take place such as:
- Endoscopic Retrograde Cholangio-Pancreatography (ERCP)- which typically reveals "beading" - alternating strictures and dilation of the bile ducts inside and outside the liver.
- Magnetic Resonance Cholangio-Pancreatography (MRCP)
Also, Most people with PSC have evidence of autoantibodies and abnormal immunoglobulin levels.
Treatment
No pharmacologic treatment has been approved for this illness yet. A trial of ursodeoxycholic acid (UDCA), a bile acid occurring naturally in small quantities in humans, as it has been shown to lower elevated liver enzyme numbers in patients with PSC and has proven effective in other cholestatic liver diseases.
Supportive treatment for PSC symptoms is the main management for this disease. These therapies are aimed at relieving symptoms such as itching with antipruritics, antibiotics to treat episodes of ascending cholangitis; and vitamin supplements, as people with PSC are often deficient in fat-soluble vitamins.
Liver transplantation is the only proven long-term treatment of PSC.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:
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