BACKGROUND AND AIMS: Autoimmune hepatitis is considered to be rare in Asia-Pacific region. There a few long term studies available. This study was planned to estimate the burden, natural history of AIH and challenges associated with management in a single non-transplant tertiary referral center. METHODS: Prospectively maintained data of patients treated as AIH was screened and patients who qualified AIH by retrospective application of simplified criteria's were enrolled. 181 patients qualified. 125 patients with substantial follow up (65 Definite AIH; 81 females; median age 46, range 8 - 79) were included in study. RESULTS: Prevalence of AIH was 1.3% and 8.74% amongst all liver disease patients and chronic liver disease respectively. 89 patients qualified as Type I AIH, 14 as type II AIH and 22 were autoimmune markers negative. Modes of presentation was acute liver failure (n = 8), chronic hepatitis (n = 17), cirrhosis (n = 89), 50 patients were decompensated), ACLF (n = 7), while 2 were clinically asymptomatic. 19 patients had preceding history of drug intake. 33 patients didn't undergo pretreatment liver biopsy. Prednisolone alone was the predominant immunosuppressive agent used, especially in decompensated cirrhotics and those with acute liver failure. First remission rates after first immunosuppression course were 60%, 85% and 63% in type I, type II and autoantibody negative groups. After a median follow up of 7 years (range 1 - 17 years), 15 patients died (12 of liver related complications) and 2 underwent liver transplantation. Failure to normalize ALT had a high hazard ratio predicting liver related death or transplantation. 11 patients had improvement on repeat liver biopsy, with 5 showing complete cirrhosis reversal. 40 patients are on long term maintenance immunosuppression. CONCLUSION: AIH, though uncommon, needs to be kept in mind as early treatment is associated with significantly good long-term prognosis.
BACKGROUND AND AIMS: Autoimmune hepatitis is considered to be rare in Asia-Pacific region. There a few long term studies available. This study was planned to estimate the burden, natural history of AIH and challenges associated with management in a single non-transplant tertiary referral center. METHODS: Prospectively maintained data of patients treated as AIH was screened and patients who qualified AIH by retrospective application of simplified criteria's were enrolled. 181 patients qualified. 125 patients with substantial follow up (65 Definite AIH; 81 females; median age 46, range 8 - 79) were included in study. RESULTS: Prevalence of AIH was 1.3% and 8.74% amongst all liver diseasepatients and chronic liver disease respectively. 89 patients qualified as Type I AIH, 14 as type II AIH and 22 were autoimmune markers negative. Modes of presentation was acute liver failure (n = 8), chronic hepatitis (n = 17), cirrhosis (n = 89), 50 patients were decompensated), ACLF (n = 7), while 2 were clinically asymptomatic. 19 patients had preceding history of drug intake. 33 patients didn't undergo pretreatment liver biopsy. Prednisolone alone was the predominant immunosuppressive agent used, especially in decompensated cirrhotics and those with acute liver failure. First remission rates after first immunosuppression course were 60%, 85% and 63% in type I, type II and autoantibody negative groups. After a median follow up of 7 years (range 1 - 17 years), 15 patients died (12 of liver related complications) and 2 underwent liver transplantation. Failure to normalize ALT had a high hazard ratio predicting liver related death or transplantation. 11 patients had improvement on repeat liver biopsy, with 5 showing complete cirrhosis reversal. 40 patients are on long term maintenance immunosuppression. CONCLUSION: AIH, though uncommon, needs to be kept in mind as early treatment is associated with significantly good long-term prognosis.