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Recurrence of primary biliary cirrhosis after liver transplantation: Histologic estimate of incidence and natural history
Open accessAbstract
The goals of this study were to determine the rate of recurrent primary biliary cirrhosis (PBC) after orthotopic liver transplantation (OLT) based on strict morphologic criteria and to evaluate histologic progression of recurrent PBC over time. Strict criteria for PBC recurrence were established as the presence of a florid duct lesion or destructive lymphocytic cholangitis within a dense portal infiltrate. Of the 784 OLTs performed at the Mayo Clinic during the first 12 years of the program, 100 met criteria for the PBC study group, and 35 met criteria for the control group. Strict histologic criteria for recurrent PBC were observed in 17 of 100 (17%) study patients (14 with florid duct lesion, 3 with destructive lymphocytic cholangitis within dense portal infiltrate). Mean follow-up for the PBC group was 4.7 years (range, 1.0 to 13.8). Mean time to recurrence was 3.7 years (median, 3.1; range, 0.3 to 7.9). In those who met strict criteria for recurrent PBC, 2 of 17 progressed to septal fibrosis (stage 3). No florid duct lesions, destructive lymphocytic cholangitis, or septal fibrosis were observed in the control group. Other less specific morphologic features of PBC (portal infiltrates, plasma cells, dense lymphoplasmacytic infiltrates, and lymphocytic cholangitis) were also evaluated in the course of this study. Based on strict criteria, a conservative histologic estimate of the rate of recurrent PBC is 17% with a mean of 4.7 years of follow-up. When criteria for histologic recurrence are expanded to include moderate lymphocytic cholangitis with lymphoplasmacytic portal infiltrate, the recurrence rate of PBC is estimated as 26%.
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“Some 20% to 30% of patients with PBC who undergo transplantation develop recurrent disease over 10 years, and up to 50% do so by 20 years of follow-up.”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [257]
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“Fortunately, recurrent PBC infrequently affects long-term patient or graft survival.(258)”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [258]
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“The median time to recurrence is 3 to 6 years.(256,257)”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [257]
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“patients on UDCA post liver transplantation appear to have lower recurrence rates compared with patients who did not receive UDCA post transplant (21% versus 62%; P = 0.004).(257)”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [257]
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“In a recent study, a higher Model for End-Stage Liver Disease score at the time of wait list registration was also associated with increased recurrence rates.(257)”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [257]
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Metadata
- Authors
- Sylvestre, P. B., Batts, K. P., Burgart, L. J., Poterucha, J. J., & Wiesner, R. H.
- Affiliations
- Mayo Clinic (US)
- Corresponding author
- Sylvestre, Pamela B.
- ORCID
- Sylvestre, Pamela B.
- Venue
- Liver Transplantation
- Published
- 2003-10
- Volume
- 9
- Issue
- 10
- Pages
- 1086-1093
- ISSN
- 1527-6465 (ISSN-L)
- OpenAlex
- W2086612029
- Cited by
- 148
- References
- 15
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 5 · 2023: 4 · 2022: 8 · 2021: 5 · 2020: 9 · 2019: 2 · 2018: 11 · 2017: 7 · 2016: 5 · 2015: 12 · 2014: 6 · 2013: 4 · 2012: 5
- FWCI
- 3.49
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Liver Transplantation (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Organ Transplantation Techniques and Outcomes
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Keywords
- primary biliary cirrhosis, recurrence, septal fibrosis, histological criteria, natural history (disease), lymphoplasmacytic infiltration
- MeSH terms
- Bile Ducts, Cholangitis, Fibrosis, Follow-Up Studies, Humans, Liver Cirrhosis, Biliary, Plasma Cells, Postoperative Complications, Recurrence, Incidence, Liver Transplantation, Disease Progression
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