LATE RECURRENT POSTTRANSPLANT PRIMARY BILIARY-CIRRHOSIS IN BRITISH-COLUMBIA

Late recurrent primary biliary cirrhosis (PBC) following orthotopic li ver transplant remains a controversial topic. The first documented cas e of recurrence occurring in 16 patients transplanted for PBC and foll owed at the authors' institution for longer than one year is presented . A 54-year-old man transplanted for PBC developed a cholestatic patte rn of enzyme elevation on pose-transplant day (PTD) 1305. Repeat antim itochondrial antibody was strongly positive (1:300 to 1:400). A liver biopsy revealed severe bile duct damage, lymphocytic cholangitis, foca l periductal noncaseating granuloma and minimal endotheliitis. Recurre nt PBC was diagnosed. At the time of orthotopic liver transplant this patient received induction immunosuppression with OKT3 crossed over to cyclosporine (CsA), azathioprine (AZA) and prednisone. AZA was discon tinued early and maintenance CsA tapered to a target trough level of 1 50 to 200 ng/mL by PTD 365. Prednisone was withdrawn by PTD 664. CsA l evels during PTDs 1225 to 1305 (before elevation of hepatobiliary enzy mes) were below target at 114 to 166 ng/mL. Of the 16 patients, all bu t three were maintained on CsA, AZA and prednisone. One was on CsA (tr ough levels on target) and AZA; the other two, including the patient w ith recurrent PBC, were on CsA only. The trough CsA level of the patie nt without recurrent PBC has been within the-target range. The authors speculate that the underlying defect in immunoregulation in PBC persi sts post-transplant and that in the patient without recurrent PBC this -defect was unmasked by lowered maintenance immunosuppression - allowi ng recurrence of PBC in a previously stable liver allograft.