Primary sclerosing cholangitis

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Primary sclerosing cholangitis
Synonyms	PSC
Pronounce	N/A
Specialty	N/A
Symptoms	Jaundice, itching, fatigue, abdominal pain
Complications	Cholangiocarcinoma, liver failure, portal hypertension
Onset	Typically between ages 30 and 40
Duration	Chronic
Types	N/A
Causes	Unknown, possibly autoimmune disease
Risks	Inflammatory bowel disease, particularly ulcerative colitis
Diagnosis	MRCP, liver biopsy
Differential diagnosis	Primary biliary cholangitis, bile duct cancer, choledocholithiasis
Prevention	N/A
Treatment	Ursodeoxycholic acid, ERCP, liver transplantation
Medication	Ursodeoxycholic acid, immunosuppressants
Prognosis	Variable, often progressive
Frequency	1 in 10,000 people
Deaths	Related to complications such as liver failure and cholangiocarcinoma

Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts which normally allow bile to drain from the gallbladder. This can lead to liver damage and eventually, liver failure.

Introduction

PSC is a chronic disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The disease affects both adults and children, but is more common in males. The pathogenesis of PSC remains unclear, but it is often associated with inflammatory bowel disease (IBD), particularly ulcerative colitis.

Causes

The exact cause of PSC is unknown, but it's thought to be related to a combination of genetic and environmental factors. The strong association between PSC and IBD suggests an autoimmune component to the disease.

Symptoms

Many people with PSC have no symptoms at all. When symptoms do occur, they may include fatigue, itching, abdominal pain, fever, and jaundice. Over time, as the liver's function decreases, symptoms of cirrhosis can develop, including fluid buildup, bleeding from enlarged veins, and confusion.

Diagnosis

Diagnosis is often made through a combination of liver function tests, imaging studies such as MRCP or ERCP, and sometimes liver biopsy. The hallmark of PSC on imaging is a pattern of strictures and dilations of the bile ducts, often described as a 'beaded' appearance.

Treatment

There's currently no known cure for PSC, and treatment is mainly supportive. This can include medication to relieve itching and antibiotics for infections. In severe cases, a liver transplant may be necessary.

Prognosis

The course of PSC is highly variable, ranging from a relatively indolent course to rapid progression to cirrhosis and its complications. PSC is a serious condition that can lead to other health problems, such as liver cirrhosis, bile duct infection, liver failure, and liver cancer.

See Also

• Liver Disease
• Liver Transplant
• Cirrhosis
• Inflammatory bowel disease
• Ulcerative colitis

References

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