Long-Term Outcome of Patients Transplanted for Primary Biliary Cirrhosis: Follow-Up > 60 Months
Murli Krishna, Denise M Harnois, Barry G Rosser, Raouf E Nakhleh. Mayo Clinic, Jacksonville, FL
Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that leads to cirrhosis requiring liver transplantation. While posttransplant survival in these patients is excellent, there is a 9-35% reported recurrence. Histologic hallmark of recurrent PBC (rPBC) is the presence of granulomatous duct injury or “florid duct lesion”. Chronic inflammation other than the florid duct lesion has also been described. The aim of this study was to characterize the clinicopathologic findings and outcome in patients transplanted for PBC at our institution, with histologic and clinical follow-up of at least 60 mo.
Design: In this retrospective review study patients were identified from the transplant database, and met the following criteria: 1) pretransplant diagnosis of PBC 2) were positive for antimitochondrial antibody, and 3) had biopsy follow-up of at least 60 mo. Patients with overlap syndrome (AIH/PBC) were excluded. Electronic medical records and relevant pathology slides were reviewed on all patients (MK). All patients received ongoing standard immunosuppression and medical treatment based on clinicopathologic findings. Histologic review included assessment for inflammation, changes of recurrent disease, fibrosis and other salient findings. rPBC was diagnosed histologically if there was presence of granulomatous duct injury. Histologic grading and staging were performed on a scale of 0-4.
Results: A total of 20 patients transplanted between 1998-2006 met the study criteria. Patient age ranged from 38-76yrs (mean 61yrs). Two patients had hepatocellular carcinoma in the explant. Two patients had failed allografts due to chronic rejection (3mo) and biliary necrosis (4mo). Histologically rPBC was present in 8 (40%) patients, first identified 8-92 mo after transplant (mean 44 mo). All cases were low stage (0-2) at the time of diagnosis. Histologic follow-up after diagnosis ranged from 0-93 mo (mean 52), and disease progression was seen in only one patient (from stage 1 to 2). 3 patients who did not have rPBC had unexplained hepatitis of grade 2 or more; with follow-up of 65-94 mo one developed stage 3 fibrosis. 19 study patients are alive with clinically stable graft function (61-153 mo, mean 100 mo); one patient died of unrelated cause at 132 months.
Conclusions: 1) rPBC occurred in 40% of our patients. 2) Recurrent disease has relatively slow histologic progression 3) Post-transplant unexplained hepatitis may represent autoimmune hepatitis in these patients. 4) Patients transplanted for PBC have an excellent clinical outcome.
Tuesday, March 20, 2012 1:00 PM
Poster Session IV # 245, Tuesday Afternoon