Type 2 autoimmune hepatitis: Long-term follow-up in single center

Autoimmune hepatitis of type 2 is a distinct type of autoimmune hepatitis with typical onset in pediatric age and, if left untreated, evolves naturally towards cirrhosis and terminal liver failure. The aim of this study is to describe and analyze a pediatric population with type 2 autoimmune hepatitis. We reviewed the medical records (clinical, biochemical and histological characteristics at diagnosis, on initial therapy and follow-up) of patients diagnosed between 1999 and 2016 and followed by the same physician. The diagnosis was confirmed by the presence of anti-LKM1 and/or anti- LC1 autoantibodies serum and by compatible histological findings. Results: We studied 19 children (15 females), median age 6.43 years (4.5 yrs SD). The most common type of onset was an isolated hypertransaminasemia (68%). Severe hepatic damage at onset was rarely found and only a patient presented cirrhosis at diagnosis. This was the only case who needed liver transplant on followup. All patient were treated by immunosuppression (standard treatment with prednisone and azathioprine 9; cyclosporine 6; cyclosporine and prednisone 1; cyclosporine and azathioprine 2; only prednisone 1). Themeanduration of follow-up was 91 months, ranging between 9 and 206 months. Relapses occurred in ten patients during discontinuation of therapy. The degree of fibrosis on liver biopsy at diagnosis correlated significantly with the number of relapses. At the end of follow-up all patients were alive; 1 patient had been transplanted; 16 patients were still treated (5 azathioprine monotherapy; 5 prednisone and azathioprine; 4cyclosporine and azathioprine; 1 cyclosporine monotherapy; 1 cyclosporine, prednisone and mycophenolate mofetil); 2 patients were free of treatment from 41 and 6 months respectively. All patients presented minor side-effects during treatment: weight gain and hirsutism in 10 patients with prednisone therapy, gingival hypertrophy in 9 patients treated with cyclosporine. All of these regressed after lowering of the drug’s dosage. One patient treated with cyclosporine manifested nephrotoxicity: renal function normalized after drug suspension. A patient with azathioprine therapy in clinical remission completed a pregnancy without complications. Conclusion: Asymptomatic hypertransaminasemia as type of onset was higher in respect to all other series reported. This onset might explain the low incidence of severe hepatic damage at diagnosis and suggests the importance of an early diagnosis in asymptomatic children with AIH-2. Immunosuppressive therapy is safe and effective in the long term.