[CLINICAL AND IMMUNOLOGICL PROFILE IN 22 PATIENTS WITH PRIMARY BILIARY CIRRHOSIS]

The primary biliary cirrhosis (PBC) is a no frequent entity in our country. Its diagnosis is based on clinical features, cholestasic pattern in liver function tests and the presence of antimitochondrial antibody in 90% of cases and other auto-antibodies in the serum, all this information is supported by characteristic hepatic histopathologic data. Also some clinical variants have been described that has suggested different denominations such as Overlap Syndrome, associated to autoimmune hepatitis and autoimmune colangitis, known by its persistent negativity of the antimitochondrials antibodies.The present study reports the clinical, biochemical and immunological findings of 22 patients with histopathologic diagnosis of PBC observed between 1994 and 1999, in Arzobispo Loayza Hospital and private offices, in Lima, Per .Results shown a cholestatic pattern in 100% of patients, jaundice in 95%, pruritus in 86%, hyperpigmentation of skin in 40.9%, hepatomegaly in 36.9% splenomegaly in 37.3% and xantelasma in 36%; alkaline phospatase was increased between 5 and 30 times the normal values in all patients, oxalacetic and piruvic transaminases were increased more than twice the normal values in 95% and 90%, respectively and hypercholesterolemia in 89% of patients; the antimitochondrial antibodies were positive in 64%, antinuclear antibodies in 32% and anti smooth muscle in 18%; 7 patients present associated autoimmune diseases, 4 with sicca syndrome, 2 with vitilige and 1 with hemolitic anemia. Three patients were classified as primary biliary cirrhosis, associated to autoimmune hepatitis; 4 patients died during the follow up and six patients were treated with ursodeoxicholic acid for more than six months.We can conclude that the profile found is a cholestasic pattern with jaundice in the majority of patients, low positivity of mitochondrial antibodies; there was no difference between AMA positive and negative patients, and in the third part of patients it was observed an associated immune disease and in three cases there was evidence of association with autoimmune hepatitis (Overlap Syndrome). Finally, the treatment with ursodeoxicolic acid improves some biochemical parameters, but apparently, they do not survival improve.