Management of Difficult Cases of Autoimmune Hepatitis

Abstract

Autoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. Current American Association for the Study of Liver Diseases (AASLD) and European Association for the Study of Liver (EASL) guidelines recommend corticosteroids alone or in combination with azathioprine as first-line treatment strategies. However, a significant proportion of patients may not be able to tolerate or achieve complete biochemical response with these options. In this article, we discuss approaches to these patients and other challenging AIH patient groups such as the asymptomatic, pregnant, elderly, and liver transplant recipients.

Keywords: Autoimmune hepatitis; Cirrhosis; Liver transplantation; Nonstandard treatment; Pregnancy; Refractory.

Figure 1

Overlap syndrome in a patient with autoimmune hepatitis, primary biliary cholangitis, and non-alcoholic steatohepatitis Liver biopsy showing findings of autoimmune hepatitis (portal and lobular inflammation with interface activity and abundant plasma cells [Figure 1A: Small window highlights plasma cells]), primary biliary cholangitis (bile duct injury with lymphocytic infiltrate [Figure 1A]), and steatohepatitis (macrovesicular steatosis, ballooned hepatocytes with Mallory bodies, and pericellular fibrosis [Figure 1A and 1B]) with bridging fibrosis (Figure 1B).