Clinical features of 89 patients with autoimmune hepatitis in Nagasaki Prefecture, Japan

We examined the clinical characteristics of 89 patients with autoimmune hep atitis (AIH) in Nagasaki Prefecture, Japan, and assessed the usefulness of a provisional scoring system for the diagnosis of AIH proposed by the Inter national Autoimmune Hepatitis Group in 1993. The majority of patients were middle-aged women in their fifties. All patients showed a hepatitic picture . Forty-three patients (48%) had an insidious or chronic onset, while 34 (3 8%) had an acute onset, and 12 (14%) had liver cirrhosis at presentation. S eventy-nine patients (89%) were positive for antinuclear antibody (ANA), an d 5 (6%) were positive for antibody to the hepatitis C virus (anti-HCV). Th e prognosis was good, with 90% 3-year survival, and most patients responded well to treatment with corticosteroids, The international scoring system w as useful for the diagnosis of AIH in most of our patients; the percentages of patients with definite and probable AIH were 48% and 47%, respectively. However, certain factors, such as negative ANA, positive antimitochondrial antibody, concurrent infection with hepatitis B or C virus, and insufficie nt response to treatment precluded the diagnosis of AIH in some patients, W hether these patients were indeed "true" AIH patients is not clear at prese nt, and further investigation of such patients may be useful for a better u nderstanding of AIH.