Staging of liver biopsy specimens from patients with chronic non-suppurative destructive cholangitis (CNDC or syndrome of primary biliary cirrhosis) has become an important part of clinical studies that are currently done in many centers. Therefore, staging methods should be based on uniform criteria that are applicable to all specimens and are easily reproducible. Most pathologists staging CNDC use the system proposed by Scheuer and modified slightly by Popper and Schaffner; and generally these methods serve well. But the features relied upon as characteristic of the earlier phases of CNDC (namely, inflammatory destruction of intrahepatic bile ducts and proliferation of ductules) are not always present in biopsy specimens from early cases, and occasionally they coexist with more advanced lesions, such as bridging necrosis.
We suggest a new staging system, based on our experience with 219 individual biopsy specimens from 101 patients with well established CNDC. Our proposed criteria are: stage I — portal hepatitis; stage II — periportal hepatitis; stage III — septal fibrosis or bridging necrosis, or both; and stage IV — cirrhosis. In most instances, we found these features easy to recognize, and one or another of them was always present. Intra-observer and interobserver variations were small. Experience with the proposed staging system indicates that stages III and IV are encountered 3 or 4 times as commonly as stages I and II. Incidence of inflammatory bile duct destruction seemed to vary little from stages I to II. Cholestasis and positive copper stains were most common in stages III and IV