Autoimmune hepatitis type 2

Orpha code: 563581OMIM code:

Definition

A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antibodies to liver kidney microsome type 1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) antibodies. The disease typically manifests in childhood or adolescence with an acute onset, often with acute liver failure. Long-term immunosuppression is usually required. Reported concurrent autoimmune diseases are autoimmune thyroiditis, diabetes mellitus, and vitiligo.

Disease data
Classification

Clinical subtype

Synonyms
AIH type 2
AIH type 2
ORPHA code
563581
OMIM code
-
ICD10 code
K75.4
ICD11 code
-

No additional description.

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