Long‐term follow‐up after recurrence of primary biliary cirrhosis after liver transplantation in 100 patients

Abstract

Orthotopic liver transplantation (OLT) is the only effective curative therapy for end-stage primary biliary cirrhosis (PBC). Survival after OLT is excellent, although recent data have shown a recurrence rate of PBC of up to 32% after transplantation. The aim of this study is to investigate the course after disease recurrence, particularly with regard to liver function and survival in a long-term follow-up. Between April 1989 and April 2003, 1,553 liver transplantations were performed in 1,415 patients at the Charité, Virchow Clinic. Protocol liver biopsies were taken after one, three, five, seven, 10 and 13 yr. One hundred (7%) patients suffered from histologically proven PBC. Primary immunosuppression consisted of cyclosporine (n = 54) or tacrolimus (Tac) (n = 46). Immediately after OLT, all patients received ursodeoxycholic acid. Corticosteroids were withdrawn three to six months after OLT. The median age of the 85 women and 15 men was 55 yr (range 25-66 yr). The median follow-up after liver transplantation was 118 months (range 16-187 months) and after recurrence 30 months (range 4-79 months). Actuarial patient survival after five, 10 and 15 yr was 87, 84 and 82% respectively. Ten patients (10%) died after a median survival time of 32 months. Two of these patients developed organ dysfunction owing to recurrence of PBC. Histological recurrence was found in 14 patients (14%) after a median time of 61 months (range 36-122 months). Patients with Tac immunosuppression developed PBC recurrence more often (p < 0.05) and also earlier (p < 0.05). Fifty-seven patients developed an acute rejection and two patients a chronic rejection episode. Liver function did not alter within the first five yr after histologically proven PBC recurrence. Multivariate analysis of the investigated patients showed that the recipient's age and Tac immunosuppression were significant risk factors for PBC recurrence. Long-term follow-up of up to 15 yr after liver transplantation, owing to PBC, in addition to maintenance of liver function, shows excellent organ and patient survival rates. Although protocol liver biopsies revealed histological recurrence in 14 (14%) patients, only two patients developed graft dysfunction. Tac-treated patients showed more frequently and also earlier histologically proven PBC recurrence; however, in our population we could not observe an impact on graft dysfunction and patient's survival.

Preventive administration of UDCA after liver transplantation for primary biliary cirrhosis is associated with a lower risk of disease recurrence

“…Recurrence of PBC is usually considered to have little impact on patient and graft survival [7]. However, graft failure, liver retransplantation or death secondary to end-stage rPBC have occasionally been reported [12,13,17,18] and thus, patients with rPBC are commonly treated with UDCA, which is associated with biochemical improvement in most cases [12]. Risk factors for rPBC have been extensively investigated in the past fifteen years [7,12,[17][18][19][20][21][22][23][24][25][26][27][28].…”

Living Donor and Deceased Donor Liver Transplantation for Autoimmune and Cholestatic Liver Diseases—An Analysis of the UNOS Database

“…2 Recently, several retrospective reviews on LT for AIH and other cholestatic liver diseases have emerged from various transplant programs. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] There are no comparisons of the long-term outcome of patients undergoing LT for each of these autoimmune diseases, and no comparative data for living donor liver transplants (LDLT) vs. deceased donor liver transplants (DDLT) for these conditions.…”

Cyclosporine A Protects Against Primary Biliary Cirrhosis Recurrence After Liver Transplantation

“…There is evidence to suggest that recurrent disease can be observed as early as 1 month following LT (3)(4)(5); however, the prevalence of recurrent PBC usually increases progressively with time and histological disease is reported in about a third of patients 10 years after LT (2,(6)(7)(8)(9)(10)(11)(12)(13). (2,(6)(7)(8)(9)(10)(11)(12)(13).…”