title: Liver transplantation for primary biliary cirrhosis: a long-term pathologic study.
abstract: Although recurrent primary biliary cirrhosis (PBC) after liver transplantation (LT) has been reported, the full spectrum of changes and progression to fibrosis and cirrhosis is not yet established. We performed a detailed retrospective clinicopathologic analysis of 43 patients who underwent LT for PBC. Eight patients (18.6%) had definite recurrent PBC with florid duct lesions, 5 patients (11.6%) had recurrence with features of autoimmune liver disease, not otherwise specified (AILD-NOS), 7 patients (16.3%) had plasmacytosis only, 4 patients (9.3%) had chronic rejection, 18 patients (41.9%) have no recurrence at present, and 1 patient (2.3%) had acquired hepatitis C. Although definite diagnoses of PBC and AILD-NOS recurrences (n = 13) were made 1 month to 14 years (median, 4 years) post-LT, all patients had plasmacytosis in their earlier biopsy specimens. Also, these patients showed similar pre-LT and post-LT clinical features, with progressive fibrosis in 4 of 8 and 2 of 5 patients, respectively. Four of 13 patients with definite recurrence and 14 of 18 patients with no recurrence were administered azathioprine (AZA) as part of their post-LT therapy (P =.01). Six of 13 and 16 of 18 patients currently are alive, with median follow-ups of 11 and 5 years, respectively. No significant differences were seen with donor-recipient group A, group B, group O blood type, sex, or HLA mismatches; native liver histological characteristics; or tacrolimus-based therapy. In conclusion, recurrent autoimmune liver disease was seen in 30% of patients after LT for PBC and had features of PBC and/or AILD-NOS. Progression seen in 46% of patients was associated with late graft failure. Patients with no recurrent disease had shorter follow-up periods and more frequent immunosuppression, including AZA; some may still develop recurrence with longer follow-up.
authors.affiliation: Department of Anatomic Pathology, Lahey Clinic Medical Center, 41 Mall Road, Burlington, MA 01805, USA. urmila.khettry@lahey.org
authors.forename: Urmila
authors.initials: U
authors.lastname: Khettry
authors.forename: Namrata
authors.initials: N
authors.lastname: Anand
authors.forename: Peter N
authors.initials: PN
authors.lastname: Faul
authors.forename: W David
authors.initials: WD
authors.lastname: Lewis
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Pomfret
authors.forename: James
authors.initials: J
authors.lastname: Pomposelli
authors.forename: Roger L
authors.initials: RL
authors.lastname: Jenkins
authors.forename: Fredric D
authors.initials: FD
authors.lastname: Gordon
mesh_terms: D001327:Autoimmune Diseases / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000473:pathology* / Q000601:surgery*; D008107:Liver Diseases / Q000276:immunology / Q000473:pathology; D016031:Liver Transplantation / Q000473:pathology*; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D014184:Transplantation, Homologous / Q000473:pathology