Seronegative autoimmune hepatitis

Orpha code: 563589OMIM code:

Definition

A form of autoimmune hepatitis characterized by the features of classic autoimmune hepatitis (i. e. clinical presentation as acute or chronic cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aspartate aminotransferase and alanine aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, therapeutic response to corticosteroids) in the absence of serum autoantibodies. Clinical manifestations include fatigue, malaise, arthralgia, jaundice, at later stages also signs of advanced chronic liver disease, such as spider nevi, caput medusae, splenomegaly, ascites, and palmar erythema. Presence of concurrent autoimmune diseases is frequently observed.

Disease data
Classification

Clinical subtype

Synonyms
Autoantibody-negative autoimmune hepatitis
Seronegative AIH
Autoantibody-negative autoimmune hepatitis
Seronegative AIH
ORPHA code
563589
OMIM code
-
ICD10 code
K75.4
ICD11 code
-

No additional description.

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