PAEDIATRIC AUTOIMMUNE LIVER DISEASE: INDICATIONS AND OUTCOME OF LIVER TRANSPLANTATION

Abstract

Autoimmune liver disease(AILD)includes sclerosing cholangitis(SC), autoimmune hepatitis type 1 & 2(AIHT1&AIHT2)and overlap syndrome(OS). Aim: To review indications and outcome of liver transplant (LT) in paediatric AILD. Methods: Retrospective review of all paediatric AILD presenting from 1981-2004. Results: 102 patients had confirmed AILD, (62 F). 66 had AIHT1, 18 AIHT2, 9 SC and 9 OS. Median age at disease onset was 11.9 yr (range 1.2-18.3). 98/102 were treated with prednisolone (PNL) and azathioprine (AZA). Table:TableMedian age at transplant was 14.3 yrs (range 1.7 to 21.8). 1 child with AIHT1 and 3 with AIHT2 had emergency LT. Median duration of treatment to LT for the remaining 15 children was 29 mo (range 8-92). Indications for LT were acute liver failure (22%), disease progression despite treatment (39%), poor quality of life (22%), complications of portal hypertension (22%) and hepatopulmonary syndrome (5%). Main postoperative complications were biliary complications4, GI bleed1 and steroid induced psychosis1. 11 patients (57%) had 19 episodes of acute rejection and 2 (11%) had chronic rejection. 5 patients (26%) had disease recurrence (1AIHT1, 3AIHT2, 1ASC). Median time from LT to disease recurrence was 27 mo (range 5-51). 4 had repeat LT (2 chronic rejection, 2 disease recurrence) and 1 had a third LT (hepatic artery thrombosis). There were 4 deaths due to sepsis1, surgery for ulcerative colitis1and disease recurrence2. Conclusion: Most paediatric AILD respond to medical therapy, only a minority require LT. Counselling for LT should indicate the morbidity and mortality related to graft rejection and disease recurrence.