Primary sclerosing cholangitis

What is it?

Primary sclerosing cholangitis (PSC) is a chronic progressive disease of unknown etiology, characterized by inflammation, fibrosis and stenosis of the medium- and large-calibre intra- and extrahepatic bile ducts, which prevents the outflow of bile that accumulates in the liver, damaging its cells. It affects more men (70%), and the average age of diagnosis is 40 years. Although the cause is unknown, PSC is associated with chronic inflammatory bowel disease (IBD), which is present in 80% of patients. About 5% of patients with ulcerative colitis (UC) and about 1% of patients with Crohn's disease (CD) develop PSC. This suggests that immune-mediated mechanisms related to genetic predisposition underlie the disease. The coexistence of inflammatory bowel disease and PSC increases the risk of colorectal carcinoma, and PSC is a known risk factor for cholangiocarcinoma.

Which are the symptoms?

The onset of symptoms is often insidious, in most cases progressive, with general malaise accompanied by itching, and jaundice (yellowish discoloration of the eyes and/or skin) usually develops later. Recurrent attacks of pain in the right upper quadrant of the abdomen, accompanied by high body temperature, occur in 10-15% of patients and are a sign of ascending bacterial cholangitis due to biliary obstruction. Steatorrhoea (fatty/unctous stools) and changes associated with deficiency of fat-soluble vitamins may also be seen.  Patients with MICI are diagnosed by changes in liver parameters and cholestasis in blood tests. PSC is usually slow and inexorably progressive, eventually leading to decompensated cirrhosis with portal hypertension, ascites and liver failure.                                                                                       

The most dangerous complication of PSC is cholangiocarcinoma, a malignancy of the biliary tract, which occurs in approximately 10-15% of patients with long-term disease.

The most common symptoms are as follows:

·         Itching

·         General malaise

·         Fever with chills

·         Abdominal pain

·         Steatorrhea

·         Jaundice

How is it diagnosed?

Diagnosis is based on haematochemical tests, where an increase in alkaline phosphatase and gamma glutamyl transpeptidase rather than hepatic enzymes (aminotransferases or transaminases) is typical. Gamma globulin levels, especially IgM, are often increased. Autoimmune antibodies (anti-nuclear and anti-neutrophil cytoplasmic antibodies) are positive in most cases. However, a cholangiogram demonstrating stenosis and subsequent multifocal dilatation of the intra- and extrahepatic bile ducts is essential for the diagnosis of PSC. Cholangiography can be obtained during abdominal MRI with cholangiographic sequences (MRCT) or during endoscopic retrograde cholagiopancreatography (ERCP), an invasive endoscopic procedure. Given its low invasiveness with comparable diagnostic accuracy, MRCP is the method of choice, and ERCP has a role in patients where biliary stenosis needs to be treated. In recent years, echoendoscopy has also become increasingly important in the study of the biliary tract.

Suggested exams

How is it treated?

Asymptomatic patients usually need only follow-up with imaging and laboratory tests.  Ursodeoxycholic acid reduces pruritus and improves biochemical markers, but not survival. Chronic cholestasis and cirrhosis require supportive therapy. Cholangitic episodes require antibiotic therapy, and if accompanied by endoscopically detected biliary tract stenosis, its treatment is attempted during ERCP. Despite the association between PSC and RCU, the two diseases usually have a separate clinical course. 
Liver transplantation is the only treatment that improves the life expectancy of patients with idiopathic primary sclerosing cholangitis and provides a cure. Recurrent bacterial cholangitis or complications of terminal hepatopathy (e.g. intractable ascites, portosystemic encephalopathy, or bleeding from esophageal varices) are reasonable indications for liver transplantation.

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