S2944 Autoimmune Hepatitis Presenting With Concomitant Chronic Pancreatitis

Abstract

Introduction: Autoimmune Hepatitis (AIH) is a progressive form of chronic hepatitis, with periods of remissions and exacerbations. Diagnosis includes abnormally high levels of immunoglobulins and multiple autoantibodies, with female predominance. Clinical presentation is variable, with a spectrum extending from asymptomatic cases to fulminant liver failure. Presenting symptoms may include abdominal pain, malaise, fatigue, and small joint arthralgia. We present a case of a 36 YO M with PMH of alcohol dependence and acute pancreatitis who was diagnosed with AIH. Case Description/Methods: A 36 YO AA M with PMH of alcohol dependence (in remission for 2 yrs), tobacco use, and pancreatitis, presented to the ED with non-radiating mid-epigastric abdominal pain 10/10 in severity, associated with NBNB emesis, exacerbated by movement for 2 days. The patient was hemodynamically stable and on exam he had icteric frenulum, abdominal distension, with liver span 12cm at mid-clavicular line, and absence of fluid wave, shifting dullness, rebound tenderness, or voluntary guarding. Labs notable for pancytopenia, elevated lipase, elevated ALP, elevated AST and ALT with 2:1 ratio, and hyperbilirubinemia. MRCP showed cirrhotic liver with splenomegaly and varices, as well as with free fluid in the lesser sac along the pancreatic head, duodenum, and right retroperitoneum (compatible with acute pancreatitis). Patient received IV fluids for pancreatitis. Additional labs were remarkable for elevated actin smooth muscle antibody at 26U (ref range: 0-19), ANA positive, with high alpha-1-antitrypsin levels and normal ceruloplasmin levels. The patient left against medical advice; and was given resources for Hepatology with referral for liver transplant (Figure). Discussion: There is limited data regarding patients with concomitant AIH and pancreatitis. Our pt presented with a AIH with secondary acute on chronic pancreatitis, in the absence of additional autoimmune manifestations. Mechanism of AIH remains poorly understood; however, there is an association between the HLA gene and AIH. Genetic studies have shown HLA-DRB1*0301 and HLA-DRB1*0401 as primary and secondary genotypes susceptible to AIH, as well as genetic variants with CARD10 and SH2B3. Products secondary to metabolism of ETOH such as alcohol dehydrogenase, malondialdehyde, and acetaldehyde, can lead to development of autoantibodies. Additional research is indicated to evaluate the relationship between AIH and acute pancreatitis.Figure 1.: A closer look at the case with images from the EGD, EUS, Right upper quadrant ultrasound and MRCP.