Child hood Autoimmune Liver Disease: Indic ations and Outc ome of Liver T ransplantatio n

Backgroun d: Graft rejection and disease recurren ce are well-recogni zed complication s of liver transplantation (LT) for autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC). We describe indications and outcome of LT for childhood AIH and AISC. Patients and Methods: Twenty-ye ar retrospective review of a cohort of children (n ¼ 101) with AIH, AISC, or AIH/scle rosing cholangitis overlap syndrome from a single center. Results: AIH type 1 (AIH1, n ¼ 67) was more common than AIH type 2 (AIH2, n ¼ 18), AISC (n ¼ 8), or overlap syndrome (n ¼ 8). Overall, 18 patients (18%) required LT, the indication s being failure of medical therapy (n ¼ 16) and fulminant liver failure (n ¼ 2). Patients with AIH who required LT had a more prolonged prothrombin time at presentation compared with those who did not undergo transplantation ( P ¼ 0.01). Patients with AIH1 who received LT had a lower aspartate transami nase ( P ¼ 0.009) and alanine transaminase ( P ¼ 0.02) levels at initial diagnosis compared with those with AIH1 who did not undergo transplantation . PostLT, 11 patients (61%) had 18 episodes of rejection, most were steroid sensitive. Disease recurrence was observed in 7 patients (39%, median duration post-LT 33 months), more common in AIH2 (80% recurrenc e rate), and those taking cyclosporine (71%, 5/7 patients) compared with those taking tacrolimus (18%, 2/11 patients; P < 0.05) and in 3 of 3 children who did not have maintenance steroids post-LT. The overall 5- and 7-year postLT survival rate was 94% and 88%, respectively. Conclusio ns: LT is a good therapeu tic option for progressive AIH and AISC, although recurrence of the primary autoimmune process limits the outcome.