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First of all, this condition is due to an inflammatory process of the bile ducts in the liver and outside of the liver. There  maybe an underlying infection, for instance some bile duct tumor or for example an underlying AIDS infection. In that latter case there are chronic infections that cause scarring and the development of cirrhosis of the liver.

Furthermore, there is another form of an autoimmune type cholangitis. The name is primary sclerosing cholangitis (PSC). In this cholangitis you frequently find there is a chronic inflammation of the bile ducts.

As a result, this causes blocking of the bile ducts, which will also cause cirrhosis of the liver. Young men who often also have ulcerative colitis harbour this congenital cholangitis. Additionally, certain cell surface antigens (the histocompatibility antigens HLA-B8, -DR3, -DR2, and -DR6) express themselves on the cell surface.


Finally, the clinical presentation can vary depending what the underlying cause of the cholangitis is. However, it must be remembered that it may take a long time to diagnose this as the onset is usually slow with symptoms of fatigue, itchy skin (=pruritus) and the development of jaundice. In general, there can be right upper abdominal pain with a fever. Additionally, if hepatitis is present, then symptoms of liver and spleen enlargement, ascites and portal hypertension with bleeding from esophageal varices can be present.


Most noteworthy, this is a chronic illness and very complex clinically. Above all, it is important to realize that a specialist such as a gastroenterologist can help with the management of the disease. Equally important, if there are bouts of recurrent bacterial cholangitis, the gastroenterologist will do a procedure called endoscopic retrograde cholangio-pancreatoscopy (ERCP study). Certainly, during the procedure the physician obtains samples of bile for culture and sensitivity testing that guide him/her as to what antibiotic to use to eradicate the infection. Even more, if strictures develop these can be dilated under ERCP guidance or a stent can be placed to overcome these strictures. In end stage disease associated with PSC a liver transplant might be the only cure.


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Last modified: August 29, 2018

This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you.