The Pathology of Primary Biliary Cirrhosis with Emphasis on Histogenesis

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Abstract
This paper deals with studies of 33 patients who had so-called primary biliary cirrhosis and a patent extrahepatic biliary tree. Of the 33 patients, 19 had cirrhosis histologically and 14 did not. Pathologically, whether evidence of cirrhosis was present or not, all biopsies revealed some form of hepatitis characterized by variable degrees of parenchymal injury, usually concentrated in the region of the limiting plate, by inflammation of the portal tracts, frequently involving necrosis and destruction of the interlobular ducts, and in most instances by bile stasis. Because necrosis of the limiting plate and the associated inflammatory reaction were usually the most striking features of this form of hepatitis, and because destruction of this zone must also lead to destruction of the cholangioles (canals of Hering, ductules), the term "cholangiolitic hepatitis" seemed an appropriate designation in most instances. Correlation of the pathologic features with the clinical data indicates that this form of hepatitis is an extremely chronic process that continues for years, with the preservation of a normal vascular pattern. When cirrhosis is present or develops later, it usually resembles that seen to occur as a result of extrahepatic obstruction of the bile ducts and is probably best designated as "cholangiolitic" or "primary biliary" cirrhosis.