Type your tag names separated by a space and hit enter

Hepatitis, Autoimmune

Hepatitis, Autoimmune is a topic covered in the 5-Minute Clinical Consult.

To view the entire topic, please or purchase a subscription.

Medicine Central™ is a quick-consult mobile and web resource that includes diagnosis, treatment, medications, and follow-up information on over 700 diseases and disorders, providing fast answers—anytime, anywhere. Explore these free sample topics:

Medicine Central

-- The first section of this topic is shown below --

Basics

Description

A chronic inflammatory disorder of unknown etiology affecting the liver; characterized by periportal necrosis (interface hepatitis or piecemeal necrosis), hypergammaglobulinemia, and autoantibody formation

  • Can be asymptomatic or lead to acute hepatic failure
  • Diagnosis of autoimmune hepatitis (AIH) is based on clinical, histologic, biochemical, and immunologic criteria.

Epidemiology

Incidence
0.1 to 1.9/100,000 per year in Caucasians. Highest incidence in Northern Europeans; lower in Japanese


Prevalence
  • 11.6 to 16.9/100,000 in Europe
  • Female > male (all types; 3.6:1)
  • Bimodal age distribution for type I: Adolescents and adults in the 4th to 6th decades are most at risk.
  • Type II is more common in Southern Europe; primarily affects young females and children

Etiology and Pathophysiology

  • Hepatic damage is cell-mediated.
    • Human leukocyte antigen (HLA) engages antigen-processing cells to stimulate cytotoxic T cell response.
    • Cytotoxic T-lymphocytes infiltrate hepatic tissue, releasing cytokines and damaging hepatocytes.
    • Chronic hepatic inflammation leads to fibrosis and cirrhosis in advanced cases.
  • Fulminant inflammation can lead to acute liver failure without cirrhosis.
  • Type I (80% of cases): circulating anti–smooth muscle antibodies (SMAs) and/or antinuclear antibodies (ANAs)
  • Type II: antibodies to liver/kidney microsome type I (anti-LKM-1)
  • Type III (least understood): soluble liver antigen/liver pancreas antigen (SLA/LP); clinically similar to type I but with different antibody profile
  • Idiopathic

Genetics
  • Associated with HLA haplotypes DR3, DR4 in white Northern Europeans (1)

Risk Factors

  • Female
  • Triggers include medications and viruses (herpes simplex, acute hepatitis A or B, Epstein-Barr, cytomegalovirus).
  • Associated autoimmune conditions

Commonly Associated Conditions

  • Type 1 diabetes mellitus
  • Autoimmune thyroiditis
  • Immune-mediated hemolytic anemia
  • Idiopathic thrombocytopenic purpura
  • Celiac sprue; ulcerative colitis
  • Rheumatoid arthritis, vitiligo
  • Primary biliary cirrhosis and primary sclerosing cholangitis occasionally overlap with AIH (1).

-- To view the remaining sections of this topic, please or purchase a subscription --

Citation

Stephens, Mark B., et al., editors. "Hepatitis, Autoimmune." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116835/all/Hepatitis__Autoimmune.
Hepatitis, Autoimmune. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116835/all/Hepatitis__Autoimmune. Accessed March 22, 2019.
Hepatitis, Autoimmune. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116835/all/Hepatitis__Autoimmune
Hepatitis, Autoimmune [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 22]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116835/all/Hepatitis__Autoimmune.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Hepatitis, Autoimmune ID - 116835 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116835/all/Hepatitis__Autoimmune PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -