A Case Report of Concomitant Acute Autoimmune Pancreatitis and Fulminant Autoimmune Hepatitis in a Young Male

Abstract

Purpose: Autoimmune hepatitis (AIH) and autoimmune pancreatitis (AIP) share the common threads of immune system dysfunction, hypergammaglobulinemia, and steroid responsiveness, but otherwise are two distinct entities with different presenting symptoms and sequelae. AIH is a chronic inflammatory disorder of unknown etiology characterized by circulating autoantibodies directed against cellular components that can progress to cirrhosis and end-stage liver disease. AIP has emerged as an increasingly recognized cause of chronic pancreatitis that is important to identify due to its dramatic response to steroid treatment. AIP typically presents with obstructive jaundice in the setting of elevated serum IgG4 levels and imaging which often reveals pancreatic duct stricturing and a pancreatic mass that can be confused with cancer. To our knowledge AIH has only been reported to coexist with AIP in three cases. Here we report the first known case of concomitant acute AIP and fulminant AIH in an otherwise healthy 28-year-old Hispanic male. The patient presented for evaluation of progressive jaundice, malaise, nausea, and migratory joint pain. Physical exam was remarkable for jaundice and multiple spider angiomata without asterixis or change in mental status. Initial laboratory findings demonstrated an ALT of 1154 IU/L, AST of 1778 IU/L, total bilirubin of 28.1 mg/dL, and an INR of 2. ANA and anti-smooth muscle antibody were also positive at titers of 1:80, while a hepatitis panel, HIV test, ceruloplasmin, and liver cytosol autoantibodies were all negative. Ultrasound demonstrated patent hepatic vasculature, heterogeneous echogenicity of the liver, and a nodular liver surface. Over the next 48 hours the patient's INR increased to 3.1 and he developed epigastric abdominal pain with a lipase equal to 785 U/L. A CT abdomen at that time showed inflammatory changes surrounding the pancreas and associated small bowel ileus. A transjugular liver biopsy was subsequently performed which revealed fulminant hepatitis with no definitive residual hepatocytes seen as well as marked ductular reaction with interspersed lymphocytes and clustered plasma cells, eosinophils, and neutrophils throughout the sample. Total IgG and IgG4 were both elevated at 2280 mg/dL and 220 mg/dL, respectively. Prednisolone therapy was initiated for concomitant fulminant AIH and acute AIP. Dramatic improvement in both clinical and laboratory parameters were seen immediately, and the patient remained asymptomatic with a normal INR and liver function tests six months later. He has since been maintained on azathioprine alone without further steroid requirement.