II. Epidemiology

  1. Primarily in young women with autoimmune disorders
  2. Prevalence: 11 to 17 in 100,000 persons

III. Associated Conditions

  1. Hypergammaglobulinemia (IgG levels up to 3x normal)

IV. Labs

  1. Transaminases Moderately elevated
    1. Alanine Aminotransferase (ALT) 150-1000
    2. Aspartate Aminotransferase (AST) 150-1000
  2. Cholestasis Labs elevated
    1. Gamma glutamyl transferase (GGT) elevated
    2. Alkaline Phosphatase
  3. Liver Function Tests abnormal
    1. Albumin depressed
    2. ProTime prolonged
    3. Bilirubin elevated

V. Diagnosis

  1. Exclude other liver disease
  2. Serum Protein Electrophoresis
    1. IgG levels 1.2 to 3 times normal
    2. High Negative Predictive Value for Autoimmune Hepatitis
  3. Specific Labs (False Negative: 10-20%)
    1. Antinuclear Antibody (ANA)
    2. Smooth Muscle Antibody (SMA)
    3. Anti-LKM Microsome type 1 Antibody

VI. Management

  1. See Prevention of Liver Disease Progression
  2. Corticosteroids
    1. Significantly improve survival

VII. Course

  1. Severe progressive liver disease is more typical

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Ontology: Hepatitis, Autoimmune (C0241910)

Definition (NCI) Hepatitis caused by autoantibodies. Drugs, infections, and toxins may trigger the production of the autoantibodies against the liver parenchyma.
Definition (MSH) A chronic self-perpetuating hepatocellular INFLAMMATION of unknown cause, usually with HYPERGAMMAGLOBULINEMIA and serum AUTOANTIBODIES.
Concepts Disease or Syndrome (T047)
MSH D019693
ICD9 571.42
ICD10 K75.4
SnomedCT 197284004, 408335007, 68784009
English Autoimmune chronic active hep, Autoimmune Hepatitides, Autoimmune Hepatitis, Hepatitides, Autoimmune, Hepatitis, Autoimmune, autoimmune hepatitis, autoimmune hepatitis (diagnosis), hepatitis chronic active autoimmune, chronic active autoimmune hepatitis, chronic active autoimmune hepatitis (diagnosis), Hepatitis autoimmune, Autoimmune Chronic Hepatitis, Hepatitides, Autoimmune Chronic, Hepatitis, Autoimmune Chronic, Autoimmune Chronic Hepatitides, Chronic Hepatitides, Autoimmune, Chronic Hepatitis, Autoimmune, Hepatitis, Autoimmune [Disease/Finding], hepatitis autoimmune, autoimmune disorder hepatitis, Autoimmune chronic active hepatitis, Autoimmune hepatitis, Autoimmune hepatitis (disorder), autoimmune; hepatitis, hepatitis; autoimmune
Dutch auto-immuun hepatitis, auto-immuun; hepatitis, hepatitis; auto-immuun, Auto-immuunhepatitis, auto-immuunhepatitis, Autoimmune hepatitis, Autoimmuunhepatitis, Hepatitis, autoimmune
German Hepatitis autoimmun, autoimmune Hepatitis, Autoimmune Hepatitis, Hepatitis, autoimmune
Spanish Hepatitis autoinmune, Hepatitis Crónica Autoinmune, hepatitis autoinmune, Hepatitis autoinmunitaria, hepatitis activa crónica autoinmune, hepatitis autoinmunitaria (trastorno), hepatitis autoinmunitaria, Hepatitis Autoinmune
Swedish Hepatit, autoimmun
Japanese ジコメンエキセイカンエン, 肝炎-自己免疫性, 自己免疫性肝炎, 自己免疫肝炎
Finnish Autoimmuunihepatiitti
Russian GEPATIT AUTOIMMUNNYI, AUTOIMMUNNYI GEPATIT, АУТОИММУННЫЙ ГЕПАТИТ, ГЕПАТИТ АУТОИММУННЫЙ
Czech Autoimunitní hepatitida, hepatitida autoimunitní, autoimunitní hepatitida, autoimunitní chronická hepatitida
Korean 자가면역 간염
Portuguese Hepatite Autoimune, Hepatite Crônica Autoimune, Hepatite auto-imune
Polish Zapalenie wątroby autoimmunologiczne
Hungarian Autoimmun hepatitis
Norwegian Autoimmun hepatitt, Kronisk autoimmun hepatitt
French Hépatite auto-immune, Hépatite autoimmune
Italian Epatite autoimmune