Autoimmune hepatitis (AIH) is an immune-mediated and inflammatory liver disorder. It is currently divided into types 1 and 2, differentiated and defined by the presence of specific autoantibodies. The objectives are to describe the prevalence and incidence of type 1 and 2 autoimmune hepatitis and to analyze the clinics, biochemical and histopathological profiles at diagnosis and follow-up, initial therapy, response to therapy and long-term follow-up in three Italian centers of patients with type 1 and type 2 AIH.
Background: Autoimmune hepatitis (AIH) is an immune-mediated and inflammatory liver disorder with typical onset in pediatric age. It is currently divided into types 1 and 2, differentiated and defined by the presence of specific autoantibodies. AIH type 1 (AIH-1) is characterised by smooth muscle and/or antinuclear autoantibodies (SMA/ANA) while anti-liver kidney microsomal (anti-LKM) or liver cytosol type 1 (anti-LC1) identified AIH type 2 (AIH-2). The conventional treatment is based on corticosteroids and azathioprine as first line therapy. The optimal duration is not clear definied, however it should be extended for at least five years due to the frequent relapses. Aims of the study: The primary objective is to describe the prevalence and incidence of type 1 and 2 autoimmune hepatitis on a selected italian pediatric population. Secondary objectives are to analyze the clinical, biochemical and histopathological profiles at diagnosis and follow-up, type of treatment received, response to therapy and long-term follow-up. Methods: The investigators conducted a retrospective and prospective cohort study on patients aged ≤ 18 years who the diagnosis of AIH 1 and 2 between 01/01/1989 and 31/12/2019 and were followed in three Italian pediatric centers.
Study Type
OBSERVATIONAL
Enrollment
109
pediatric patients with clinical, laboratory (transaminase and IgG increase), instrumental (ultrasound, liver biopsy) characteristics
University Hospital of Ferrara
Cona, Ferrara, Italy
Prevalence and incidence
The primary objective was to calculate the proportion of AIH type 1 and 2 patients present in the Italian pediatric population at a given time (prevalence) and the proportion of new AIH type 1 and 2 patients in the Italian pediatric population (0-18 years) in a given period of time (incidence).
Time frame: from 01/01/1989 to 31/12/2019
Characteristics of AIH in pediatric patients
Describe the baseline characteristics (age of onset, clinical-laboratory, radiological and histological characteristics), the response to therapy (medical and / or the possible use of liver transplantation) and the follow-up of patients with autoimmune hepatitis type 1 and 2.
Time frame: from 01/01/1989 to 31/12/2019
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