Childhood autoimmune liver disease: indications and outcome of liver transplantation.
BACKGROUND
Graft rejection and disease recurrence are well-recognized complications 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.


METHODS
Twenty-year retrospective review of a cohort of children (n = 101) with AIH, AISC, or AIH/sclerosing cholangitis overlap syndrome from a single center.


RESULTS
AIH type 1 (AIH1, n = 67) was more common than AIH type 2 (AIH2, n = 18), AISC (n = 8), or overlap syndrome (n = 8). Overall, 18 patients (18%) required LT, the indications being failure of medical therapy (n = 16) and fulminant liver failure (n = 2). Patients with AIH who required LT had a more prolonged prothrombin time at presentation compared with those who did not undergo transplantation (P = 0.01). Patients with AIH1 who received LT had a lower aspartate transaminase (P = 0.009) and alanine transaminase (P = 0.02) levels at initial diagnosis compared with those with AIH1 who did not undergo transplantation. Post-LT, 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% recurrence 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 post-LT survival rate was 94% and 88%, respectively.


CONCLUSIONS
LT is a good therapeutic option for progressive AIH and AISC, although recurrence of the primary autoimmune process limits the outcome.
Chai
Pei Fan
PF
Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.
Lee
Way Seah
WS
Brown
Rachel M
RM
McPartland
Jo L
JL
Foster
Katharine
K
McKiernan
Patrick J
PJ
Kelly
Deirdre A
DA
D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D002648:Child; D015209:Cholangitis, Sclerosing / Q000276:immunology / Q000601:surgery*; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000201:enzymology; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000276:immunology / Q000401:mortality; D008297:Male; D011517:Prothrombin Time; D012008:Recurrence; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D016019:Survival Analysis; D015996:Survival Rate; D013577:Syndrome; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D016559:Tacrolimus