Autoimmune hepatitis in India: single tertiary referral centre experience

Abstract

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.

Presence and type of decompensation affects outcomes in autoimmune hepatitis upon treatment with corticosteroids

“…In our study, 70% patients of the whole cohort had cirrhosis at presentation, with 45% of these patients having clinical evidence of decompensation. This proportion of cirrhosis at presentation is similar to previously described cohorts of AIH from this region and is higher than those described from the West 2,3,9,10,18,20,29 . In addition, direct presentation with decompensated cirrhosis is uncommonly described from western cohorts.…”

“…demonstrated the reversal of clinical decompensations in 62% of patients with limited information on other treatment‐related outcomes, such as biochemical remission 19 . Amarapurkar et al ., in their study, demonstrated biochemical remission in 68% of these patients, 18 a rate much higher than the present study. Notable differences with the present study included the lack of histological confirmation and detailed algorithm in the management of these patients.…”

“…A subset of patients with AIH and cirrhosis have decompensated disease at presentation 18–21 . Advanced disease, intolerable adverse events with drugs, and increased risk of infections often preclude immunosuppression in these patients 7,16,22 .…”

Retrospective, single-center analysis of autoimmune hepatitis in Jordanian children: clinical features, treatments, and outcomes

“…Regular, lifelong follow-ups are essential post-treatment, even after remission maintenance. Studies in Egypt, Saudi Arabia, and India have reported varying remission rates and relapse tendencies [ 32 – 34 ]. In this study, 15 patients received prednisolone with azathioprine; two switched to mycophenolate due to azathioprine resistance.…”

The Asian Pacific Association for the Study of the Liver clinical practice guidance: the diagnosis and management of patients with autoimmune hepatitis

“…Biochemical resolution is defined as reduction of transaminase and IgG levels to normal. A clinical study from India [ 207 ] showed early treatment coincides with favorable long-term prognosis and failure to normalize alanine aminotransferase is a risk factor predicting disease related mortality or transplantation. A retrospective study demonstrated that more than 50% patients who achieved transaminase level below twice ULN still underwent histological progress [ 208 ].…”