Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder with an etiology that remains unclear. The pathogenesis may be a result of alterations in immune tolerance, a genetic predisposition and environmental conditions, which in collaboration induce a T-cell-mediated attack on liver antigens, leading to necro-inflammation and liver damage. AIH has marked female predilection. AIH can present at all ages, but the two peak ages of incidence are in childhood or adolescence and at around 40 years of age. Trademark biochemical/serological characteristics of AIH are elevated aminotransferase levels, positivity for autoantibodies and increased IgG. A histological picture of interface hepatitis is typical of AIH. The value of immunosuppressive therapy with glucocorticoids, alone or in combination with azathioprine, was well documented in numerous studies of AIH.
Category
Immune system disease; Digestive system disease
Brite
Human diseases [BR:br08402]
Immune system diseases
Allergies and autoimmune diseases
H01685 Autoimmune hepatitis
Digestive system diseases
Liver diseases
H01685 Autoimmune hepatitis
Human diseases in ICD-11 classification [BR:br08403]
13 Diseases of the digestive system
Diseases of liver
DB96 Autoimmune liver disease
H01685 Autoimmune hepatitis
Comment
Patients seropositive for ANA and/or anti-smooth muscle autoantibodies (SMA) have AIH type-1 whereas those presenting with positivity for anti-liver kidney type-1 autoantibody (anti-LKM-1) or anti-liver cytosol type-1 (anti-LC-1) have AIH type-2.
Other DBs
ICD-11:
DB96.0
ICD-10:
K75.4
MeSH:
D019693
Reference
PMID:27729952
Authors
Liberal R, Grant CR
Title
Cirrhosis and autoimmune liver disease: Current understanding.
Journal
World J Hepatol 8:1157-1168 (2016) DOI:10.4254/wjh.v8.i28.1157
Reference
PMID:15954109
Authors
Feld JJ, Dinh H, Arenovich T, Marcus VA, Wanless IR, Heathcote EJ
Title
Autoimmune hepatitis: effect of symptoms and cirrhosis on natural history and outcome.
Journal
Hepatology 42:53-62 (2005) DOI:10.1002/hep.20732
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