- 作者：Paolo Muratori ; Aless ; ro Granito ; Chiara Quarneti ; Silvia Ferri ; Rita Menichella ; Fabio Cassani ; Georgios Pappas ; Francesco B. Bianchi ; Marco Lenzi ; Luigi Muratori
- 关键词：Liver ; Autoimmunity ; Therapy
- 刊名：Journal of Hepatology
- 出版年：2009
- 出版时间：June 2009
- 年：2009
- 卷：50
- 期：6
- 页码：1210-1218
- 全文大小：139 K
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.