Autoimmune hepatitis in Italy: The Bologna experience

• Published in: Journal of hepatology 2009-06, Vol.50 (6), p.1210-1218
• Main Authors: Muratori, Paolo, Granito, Alessandro, Quarneti, Chiara, Ferri, Silvia, Menichella, Rita, Cassani, Fabio, Pappas, Georgios, Bianchi, Francesco B, Lenzi, Marco, Muratori, Luigi
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age Factors; Autoimmunity; Biological and medical sciences; Child, Preschool; Female; Gastroenterology and Hepatology; Gastroenterology. Liver. Pancreas. Abdomen; Hepatitis, Autoimmune - classification; Hepatitis, Autoimmune - drug therapy; Hepatitis, Autoimmune - immunology; Hepatitis, Autoimmune - pathology; HLA-DR Antigens - genetics; HLA-DRB1 Chains; Humans; Immunosuppressive Agents - therapeutic use; Italy; Liver; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Middle Aged; Other diseases. Semiology; Prognosis; Sex Characteristics; Steroids - therapeutic use; Therapy; Treatment Outcome; Young Adult
• ISSN: 0168-8278; 1600-0641
• DOI: 10.1016/j.jhep.2009.01.020

Background/Aims Autoimmune hepatitis affects mainly women. It is subdivided into type 1 and type 2 according to the autoantibody profile and without immunosuppression usually evolves to cirrhosis and end-stage liver failure. Methods We evaluated clinical, biochemical, immunological and genetic features and treatment response of 163 consecutive Italian patients with autoimmune hepatitis. Results At diagnosis, type 1 autoimmune hepatitis showed more inflamed liver histology and more pronounced cholestasis, whereas type 2 was more common in children. Male and female patients shared similar clinical, biochemical and immunological features. Of 89 patients with 5-year follow-up or longer, 23 patients irrespective of presenting clinical, biochemical and immunological features achieved complete remission (normal transaminases and gammaglobulin levels) which was maintained with minimal steroid dosage; attempt at treatment withdrawal led to disease exacerbation. Complete responders had more often HLA DRB1 ∗ 0401 ( p = 0.011) and their risk of disease progression was lower ( p < 0.0001). Conclusions Type 1 and type 2 autoimmune hepatitis is one and the same disease. Autoimmune hepatitis has similar features in male and female patients. HLA DRB1 ∗ 0401 positive patients are more likely to achieve complete remission. Continuous low-dose steroids are necessary to maintain remission, significantly reducing the risk of disease progression.