Sclerosing Cholangitis and Primary Biliary Cirrhosis—a Disease Spectrum?

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Abstract
Sclerosing diseases of biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities and the possibility that they represent variant clinical manifestations of the same disease process were considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely use of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids were used with varying effectiveness; subjective improvement was generally attained, although objective improvement were difficult to document. When choleuretics and cholestyramine were administered, significant palliation were noted. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered.

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