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Critical Reviews™ in Immunology
IF: 1.352 5-Year IF: 3.347 SJR: 1.022 SNIP: 0.55 CiteScore™: 2.19

ISSN Print: 1040-8401
ISSN Online: 2162-6472

Critical Reviews™ in Immunology

DOI: 10.1615/CritRevImmunol.2017017868
pages 407-428

Autoimmune Hepatitis: Factors Involved in Initiation and Methods of Diagnosis and Treatment

Gianfranco Lauletta
Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy
Sabino Russi
Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy
Fabio Pavone
Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy
Andrea Marzullo
Pathology Section, Department of Emergency and Organ Transplantation, University of Bari Medical School, Bari, Italy
Marilina Tampoia
Laboratory of Clinical Pathology, University Hospital, Bari, Italy
Domenico Sansonno
Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy
Franco Dammacco
Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy

ABSTRACT

Autoimmune hepatitis is an acute or mostly chronic liver disease that can affect both adults and children and has a clear prevalence for the female sex. A definite etiology has not been established, but it is known that genetic predisposing profiles and exogenous trigger factors are involved. The main diagnostic criteria include typical histological features, the occurrence of serum auto-antibodies, and increased levels of transaminases and gamma-globulins. Instances of autoimmune hepatitis sharing features with other autoimmune liver diseases have also been observed. An imbalance of the immune system with persistent activation of effector T cells has been emphasized to account for the sustained liver injury. Clinical manifestations are variable both at presentation and throughout the course of the disease, ranging from an asymptomatic state or the occurrence of non-specific symptoms to the features of end-stage liver disease such as jaundice, ascites, and gastrointestinal bleeding. A clinical and biochemical remission is achieved in at least 80% of patients receiving corticosteroids with or without the addition of azathioprine. Alternative therapeutic schedules have been proposed for unresponsive and intolerant patients. Given that relapse often occurs after therapy withdrawal, maintenance treatment is usually required.


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