Clinical manifestations of autoimmune liver diseases in Korea / 대한내과학회지

ABSTRACT

BACKGROUND: Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC), and autoimmune cholangiopathy (AIC) are major classes of liver diseases currently considered autoimmune. We investigated the clinical, biochemical, and immunologic findings in Korean patients with these disease entities. METHODS: We retrospectively analyzed 47 patients with AIH, 27 with PBC, 3 with Overlap syndrome, 15 with AIC, and 5 with PSC. RESULTS: About 90% of the patients were women, while 40% in PSC. The mean age of onset ranged from 47 to 57 years, while it was 28 years in PSC. Fatigue and jaundice were the most frequent manifestations at diagnosis. In AIH, 13% of patients had an acute onset and 1 died of fulminant hepatic failure. The serum level of IgG was elevated in 67.5%, and antinuclear antibody and anti-smooth muscle antibody were detected in 95.7% and 43.2%. Fifty eight per cent of the patients who received immunosuppressive therapy showed a complete response. In patients with PBC, the level of IgM was elevated in 75% and all of the patients were positive for anti-mitochondrial antibody. Biochemical remission was observed in 37% on ursodeoxycholic acid therapy. Except for the negative anti-mitochondrial antibody, the clinical and biochemical features in AIC were not different from those in PBC. CONCLUSION: Autoimmune liver diseases are not rare in Korea. Considering that these entities are potentially treatable diseases, a high index of suspicion is needed, especially in patients negative for the serologic markers of viral hepatitis.