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Based on 28 histologically well-established cases of chronic non-suppurative destructive cholangitis, clinico-pathological aspects of differential diagnosis and staging are reported. In only half of the cases could the diagnosis be made on the first liver biopsy. In 14 cases, two or more liver biopsy specimens were necessary for diagnosis. The most frequent and most difficult differential diagnostic issue was to distinguish between chronic non-suppurative destructive cholangitis and chronic aggressive hepatitis of viral or autoimmune origin. The histochemical demonstration of copper by the rhodanine method was of particular value for differentiating between chronic non-suppurative destructive cholangitis and chronic aggressive hepatitis as well as for diagnosis of mixed types. In two cases of mixed type, the HBs-Antigen in ground glass hepatocytes and copper-associated protein in periportal hepatocytes could be demonstrated simultaneously with Shikata's Orcein-staining. Compared with the previously used system (Scheuer 1967) the staging concept proposed by Ludwig et al. (1978) has proved to be more useful and easier to apply. This system permits recognition of the stage on routinely obtained specimens regardless of specific differential diagnostic features. Most liver biopsy specimens were assigned to stage III. Features considered characteristic of the earlier phases (inflammatory bile duct destruction and granulomas) frequently coexist with more advanced lesions in late stage.

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