Autoimmune hepatitis type 1

Orpha code: 563576OMIM 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 antinuclear antibodies (ANA), anti-smooth muscle antibodies (anti-SMA), and/or anti-soluble liver antigen/liver pancreas antigen antibodies (anti-SLA/LP). The disease predominantly develops at a post-pubertal age and most commonly takes a chronic course, although acute or acute severe presentation may also be observed. Typical concurrent autoimmune diseases are autoimmune thyroiditis and rheumatic diseases.

Disease data
Classification

Clinical subtype

Synonyms
AIH type 1
AIH type 1
ORPHA code
563576
OMIM code
-
ICD10 code
K75.4
ICD11 code
-

No additional description.

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