Multiple Faces Of Autoimmune Hepatitis- A Case Series

Abstract

Introduction- Autoimmune liver diseases are mainly of three types: autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Out of these three diseases, AIH is the most frequent, with a prevalence of 17 cases per 100,000 persons, followed by PBC and PSC. AIH and PBC mainly occur in women during menopause, AIH can also affect children and young adults. PSC mainly affects men from 20 to 40 years of age. In most cases, the three diseases can be reliably distinguished by serological analysis. However, overlap syndromes may occur in which patients present symptoms of two autoimmune liver diseases. The detection of specific autoantibodies allows precise differentiation between autoimmune liver diseases and infectious, toxic and other forms of hepatitis. AIH is often associated with chronic inflammatory rheumatic systemic diseases such as rheumatoid arthritis, Sjögren’s syndrome and systemic lupus erythematosus. AIH usually present as chronic liver disease but in 20-30% of patients as acute hepatitis or even fulminant hepatic failure.

Case Series We report case series of three females who presented differently- one presented as acute hepatitis, second one as unexplained transaminitis and third one as chronic liver disease. All were proven on biochemical tests, serology and liver biopsy. All three had successful outcome with treatment.  Our case series of three patients have different presentations- one presented as severe acute hepatitis, second one as unexplained transaminitis, and third one as cirrhotic. Moreover, cirrhotic one became pregnant and had successful outcome, like other two on timely starting of treatment and continuing as per scientific rationale. Many patients of acute severe hepatitis can land in fulminant hepatic failure but urgent treatment prevents the same, as occurred in our case. Second inference from our case series is that need of immunosuppressive decreases in pregnancy due to it being a immunocompromised state and once post-partum is over, then due to hormonal changes, need of immunosuppressive comes back and same happened in our case. Another inference from our case series is that before labelling any patient as MASH, autoimmune liver disease must be ruled out.

Conclusion- Autoimmune hepatitis can have wide spectrum of presentation like Wilson’s diseases and it varies from asymptomatic stage with mild unexplained transaminitis to acute hepatitis, fulminant liver failure and cirrhosis.