This condition is due to an inflammatory process of the bile ducts in the liver and outside of the liver. There could be an underlying infection, some bile duct tumor or underlying AIDS infection. In that latter case there are chronic infections that cause scarring and the development of cirrhosis of the liver. There is another form of an autoimmune type cholangitis, called primary sclerosing cholangitis (PSC) where there is a chronic inflammation of the bile ducts.
This causes blocking of the bile ducts, which will also cause cirrhosis of the liver. This is associated with certain cell surface antigens (the histocompatibility antigens HLA-B8, -DR3, -DR2, and -DR6) and is found in young men who often also have ulcerative colitis.
The clinical presentation can vary depending what the underlying cause of the cholangitis is. However, 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. There can be right upper abdominal pain with a fever. If hepatitis is present, then symptoms of liver and spleen enlargement, ascites and portal hypertension with bleeding from esophageal varices can be present.
As this is a chronic illness and very complex clinically, a specialist such as a gastroenterologist should be involved in the management of the disease. If there are bouts of recurrent bacterial cholangitis, samples of bile via a procedure called endoscopic retrograde cholangio-pancreatoscopy (ERCP study, thanks to www.medgadget.com for image) for culture and sensitivity testing will guide the gastroenterologist as to what antibiotic to use to eradicate the infection. If strictures develop these can be dilated under ERCP guidance or a stent can be placed to overcome strictures. In end stage disease associated with PSC a liver transplant might be the only cure.
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