Updated: 5/27/2019

Autoimmune Hepatitis

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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)
        • most common
        • + antinuclear antibodies (ANA)
        • + smooth muscle antibodies (SMA)
      • type 2 AIH
        • + liver/kidney microsome type 1 antibody (antiLKM1)
        • + antimitochondrial antibody (AMA)
  • 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
  • Pathogenesis
    • T-cell mediated autoimmune disease
  • Associated conditions
    • other autoimmune diseases
      • thyroid disease
      • ulcerative colitis
      • celiac disease
  • Prognosis
    • relapse is common
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
        • type 1 AIH
          • ANA
          • SMA
        • type 2 AIH
          • antiLKM1
          • AMA
      • liver function tests
        • ↑ transaminases
        • ↑ alkaline phosphatase
      • serum protein electrophoresis (SPEP)
        • hypergammaglobulinemia
      • liver biopsy
        • most accurate test
        • bridging or multilobular necrosis, inflammation, and fibrotic changes
        • may show cirrhosis
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
        • transaminitis > 10x normal
        • histology showing bridging necrosis or multilobular necrosis
    • azathioprine            
      • indication
        • often used alongside steroids
  • Second line
    • other immunosuppressants
      • drugs
        • mycophenolate
        • cyclosporine
        • tacrolimus
    • liver transplant
      • indication
        • disease refractory to medical therapy
Complications
  • Cirrhosis
  • Hepatocellular carcinoma
 

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