Snapshot A 24-year-old woman with a past medical history of vitiligo presents to the emergency room for abdominal pain. She has had abdominal pain intermittently for the past few weeks and has seen her primary care physician for this issue. On exam, she is slightly jaundiced with scleral icterus and has hepatosplenomegaly. Viral serologies are negative. Labs are significant for elevated transaminitis and positive smooth muscle antibody. She is started on prednisone. Introduction Clinical definition autoimmune chronic inflammation of the liver type 1 AIH (autoimmune hepatitis) login to view 3 more bullets type 2 AIH login to view 2 more bullets Associated conditions other autoimmune diseases thyroid disease ulcerative colitis celiac disease Epidemiology Demographics female > male type 1 AIH young adults type 2 AIH children Risk factors viral hepatitis measles medications may trigger a relapse Genetics AIRE gene mutation ETIOLOGY Pathogenesis T-cell mediated autoimmune disease Presentation Symptoms and physical exam jaundice abdominal pain encephalopathy pruritus hepatomegaly splenomegaly ascites Studies Diagnostic testing diagnostic approach diagnostic testing must rule out viral hepatitis studies serum antibodies login to view 6 more bullets liver function tests login to view 2 more bullets serum protein electrophoresis (SPEP) login to view 1 more bullet liver biopsy login to view 3 more bullets Differential Viral hepatitis distinguishing factor positive serologies for hepatitis viruses Hemochromatosis distinguishing factor also presents with restrictive cardiomyopathy, darkening skin, joint pain, and pancreatic insufficiency Treatment First-line steroids indication login to view 2 more bullets azathioprine indication login to view 1 more bullet Second line other immunosuppressants drugs login to view 3 more bullets liver transplant indication login to view 1 more bullet Complications Cirrhosis Hepatocellular carcinoma Prognosis Relapse is common