Liver transplantation for primary biliary cirrhosis: a long-term pathologic study.
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.
Khettry
Urmila
U
Department of Anatomic Pathology, Lahey Clinic Medical Center, 41 Mall Road, Burlington, MA 01805, USA. urmila.khettry@lahey.org
Anand
Namrata
N
Faul
Peter N
PN
Lewis
W David
WD
Pomfret
Elizabeth A
EA
Pomposelli
James
J
Jenkins
Roger L
RL
Gordon
Fredric D
FD
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