Primary Sclerosing Cholangitis

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ERCP is the preferred method for visualizing the biliary tree the network of biliary ducts.

Primary Sclerosing Cholangitis

Your doctor will find many strictures narrowing and dilations opening , which gives the duct its characteristic beaded appearance. Magnetic resonance imaging MRI may be useful in detecting blockages. An MRI uses powerful magnetic waves to create a detailed image of the inside of your body. There are a number of different treatment options for PCS, ranging from medication to surgery. Learn more about treatment for primary sclerosing cholangitis at Johns Hopkins.

See illustration: Technique of endoscopic biliary sphincterotomy illustration. Skip Navigation. Health Home Conditions and Diseases. Symptoms include: Jaundice yellowing of the skin and whites of the eyes Itching Pain in the right upper part of the abdomen Fever, chills Unexplained weight loss Fatigue PSC Diagnosis at Johns Hopkins A diagnosis of PSC begins with a comprehensive physical exam, during which you describe your symptoms and medical history.

Other diagnostic procedures include: Laboratory tests Liver biopsy Endoscopic retrograde cholangiopancreatography Magnetic resonance cholangiography Laboratory Tests Blood tests will be ordered to evaluate your liver function. The primary physiological function of bile is to assist in the breakdown and absorption of fat in the intestinal tract; a relative deficiency of bile can lead to fat malabsorption and deficiencies of fat-soluble vitamins A, D, E, K.

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Liver enlargement is seen due to portal hypertension caused by compression of portal veins by the proximate sclerosed intrahepatic bile ducts, and leads to right upper quadrant abdominal pain. PSC is generally diagnosed on the basis of having at least two of three clinical criteria after secondary causes of sclerosing cholangitis have been ruled out:. Currently, the preferred option for diagnostic cholangiography, given its non-invasive yet highly accurate nature, is magnetic resonance cholangiopancreatography MRCP , a magnetic resonance imaging technique.

MRCP has unique strengths, including high spatial resolution, and can even be used to visualize the biliary tract of small animal models of PSC.

Most people with PSC have evidence of autoantibodies and abnormal immunoglobulin levels. Other markers which may be measured and monitored are a complete blood count , serum liver enzymes , bilirubin levels usually grossly elevated , kidney function , and electrolytes. Fecal fat measurement is occasionally ordered when symptoms of malabsorption e. The differential diagnosis can include primary biliary cholangitis formerly referred to as primary biliary cirrhosis , drug-induced cholestasis , cholangiocarcinoma , IgG4-related disease , post-liver transplantation non-anastomotic biliary strictures, [21] and HIV -associated cholangiopathy.

The subgroups of PSC include the following: [1].

Etiopathophysiology

No pharmacologic treatment has been approved by the U. Some experts recommend 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. However, UDCA has yet to be shown to clearly lead to improved liver histology and survival. Supportive treatment for PSC symptoms is the cornerstone of management.

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These therapies are aimed at relieving symptoms such as itching with antipruritics e. ERCP and specialized techniques may also be needed to help distinguish between a benign PSC stricture and a bile duct cancer cholangiocarcinoma.


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Liver transplantation is the only proven long-term treatment of PSC. Indications for transplantation include recurrent bacterial ascending cholangitis, decompensated cirrhosis, hepatocellular carcinoma , hilar cholangiocarcinoma, and complications of portal hypertension.

Primary Sclerosing Cholangitis - Symptoms, Causes, and Treatment

Not all patients are candidates for liver transplantation, and some will experience disease recurrence afterward. Estimated median survival from diagnosis until liver transplant or PSC-related death is A serum alkaline phosphatase less than 1. The development of any of the cancers associated with PSC predicts a poor prognosis. Various forms of gallbladder disease such as gallstones and gallbladder polyps are also common in those with PSC. There is a male-to-female predilection in primary sclerosing cholangitis.


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  • What is primary sclerosing cholangitis?;

There is relatively little data on the prevalence and incidence of primary sclerosing cholangitis, with studies in different countries showing annual incidence of 0. Although there is no curative treatment, several clinical trials are underway that aim to slow progression of this liver disease. From Wikipedia, the free encyclopedia. Primary sclerosing cholangitis Cholangiogram of primary sclerosing cholangitis. At the present time, the exact cause is unknown.

However, research has demonstrated that genetic changes play a role in this condition because it often occurs in several members of a family. Immediate family members of someone with primary sclerosing cholangitis have an increased risk of developing the condition.

Primary sclerosing cholangitis (PSC)

Viruses that are known to cause hepatitis have not been associated with primary sclerosing cholangitis. In many cases, PSC occurs in association with inflammatory bowel disease. This suggests that a common cause for both diseases may exist. This most often occurs in children and young adults. The course of primary sclerosing cholangitis is unpredictable and not well understood.

A person can have the disease for many years before symptoms develop. They may include:. Endoscopic retrograde cholangiopancreatography ERCP combines the use of x-rays and an endoscope, which is a long, flexible, lighted tube. Through the endoscope, the physician can see the inside of the stomach and duodenum, and inject dyes into the ducts in the biliary tree and pancreas so they can be seen on x-rays. With improvements to magnetic resonance techniques, this non-invasive way to diagnose PSC is being used more frequently. Presently, there is no known specific treatment for primary sclerosing cholangitis.