Abstract
Background Autoimmune hepatitis (AIH) is a progressive inflammatory fribrosing disease of the liver of unknown etiology, which in its normal progress leads to cirrhosis. It is characterized by the increase in aminotranferases and hypergammaglobulinemia, and the presence of nonorgan and specific liver autoantibodies in serum, which allow for its classification. Two subtypes are differentiated: Type I AIH with anti-smooth muscle antibodies (SMA) and/or antinuclear antibodies (ANA); and Type II AIH with antimichondrial antibodies (LKM). AIH can be associated with other autoimmune illnesses such as thyroiditis, Type I diabetes, thrombocytopenia, hemolytic anaemia and ulcerative colitis. Likewise, the association to connective tissue diseases (CTD) has been communicated, such as Systemic Lupus Erythematosus, Sjogren’s Syndrome, Undifferentiated Connective Tissue Disease, Mixed Connective Tissue Disease, Localized Sclerodermia and Juvenile Idiopathic Arthritis.
Highlights
Autoimmune hepatitis (AIH) is a progressive inflammatory fribrosing disease of the liver of unknown etiology, which in its normal progress leads to cirrhosis
It is characterized by the increase in aminotranferases and hypergammaglobulinemia, and the presence of nonorgan and specific liver autoantibodies in serum, which allow for its classification
Type I AIH diagnosis occurred in 18/20 patients (90%), 5 males (27.8%) and 13 females (72.2%), their average diagnosis age being 9.8 yrs (Min 1.4 yrs-Max 15.2 yrs), and Type II AIH diagnosis occurred in 2/20 patients (10%), 1 male and 1 female
Summary
Autoimmune hepatitis (AIH) is a progressive inflammatory fribrosing disease of the liver of unknown etiology, which in its normal progress leads to cirrhosis. It is characterized by the increase in aminotranferases and hypergammaglobulinemia, and the presence of nonorgan and specific liver autoantibodies in serum, which allow for its classification. AIH can be associated with other autoimmune illnesses such as thyroiditis, Type I diabetes, thrombocytopenia, hemolytic anaemia and ulcerative colitis. The association to connective tissue diseases (CTD) has been communicated, such as Systemic Lupus Erythematosus, Sjögren’s Syndrome, Undifferentiated Connective Tissue Disease, Mixed Connective Tissue Disease, Localized Sclerodermia and Juvenile Idiopathic Arthritis
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