FEATURES OF AUTOIMMUNE HEPATITIS IN PRIMARY SCLEROSING CHOLANGITIS - AN EVALUATION OF 114 PRIMARY SCLEROSING CHOLANGITIS PATIENTS ACCORDINGTO A SCORING SYSTEM FOR THE DIAGNOSIS OF AUTOIMMUNE HEPATITIS

Overlapping features between primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AM) have previously been noted, To assess system atically similarities between these disorders, we have evaluated 114 P SC patients (36 women; 78 men), all confirmed by endoscopic retrograde cholangiography (ERC), according to a scoring system proposed by The International Autoimmune Hepatitis Group for the diagnosis of AIH. The scoring system attributes positive or negative scores to the paramete rs sex, ratio of elevation of serum levels of alkaline phosphatase (AL P) vs, aminotransferase, serum levels of immunoglobulins and autoantib odies, viral markers, history of drug and alcohol intake, genetic fact ors, liver histology, and response to therapy, Two of the PSC patients (2%) obtained scores above 15 before treatment, satisfying the diagno stic criterion of ''definite'' AM, Thirty-eight patients (33%) scored between 10 and 15 points and could be classified as ''probable'' AM, T he serum level of immunoglobulin G (IgG) was elevated in 68 patients ( 61% of 111 cases tested), and positive titers of antinuclear antibodie s (ANA) or smooth muscle antibodies (SMA) were detected in 24 patients (22% of 111 cases tested), Thirty-five of the PSC patients (33% of 10 5 evaluable biopsy specimens) obtained positive scores for histologica l features similar to those of AIH, but the total score for histology was in the negative range in 72 patients (69%) because of the presence of biliary changes, The frequent finding of high scores in PSC patien ts underlines the similarities PSC may have with AIH. A modification o f the scoring system, in particular by increasing the negative score f or histological biliary changes, would improve its potential to discri minate between AIH and PSC.