Liver Transplantation for Primary Sclerosing Cholangitis

A Long-Term Clinicopathologic Study

Urmila Khettry, Andrew Keaveny, Atoussa Goldar-Najafi, W. David Lewis, Elizabeth A. Pomfret, James J. Pomposelli, Roger L. Jenkins, Fredric D. Gordon

Research output: Contribution to journalArticle

94 Citations (Scopus)

Abstract

The course and outcome of patients after liver transplantation (LT) for primary sclerosing cholangitis (PSC) are still debated. Our purpose is to define retrospectively, the post-LT clinicopathologic findings seen in 51 PSC patients with a follow-up of 2 to 14 years. Of the total 51 patients, 16 with native liver hilar xanthogranulomatous cholangiopathy (XGC) had median graft and patient survival of 573 and 835 days, respectively compared with 2489 and 2794 days, respectively, in 35 patients without XGC. Perioperative complications resulted in 9 early deaths (day 0 to 52). Of the remaining 42 patients, 6 had recurrent PSC (R-PSC) with typical histologic and cholangiographic findings, 12 had autoimmune liver disease-not otherwise specified with histology of autoimmune hepatitis/overlap syndrome, 3 had chronic rejection, 4 had ischemic cholangiopathy, and 17 had no recurrence. The presence of inflammatory bowel disease, total ischemia time of ≥11 hours, recipient-donor ABO and HLA Class I and II matches, and the type of immunosuppression did not affect the post-LT outcome. Recipient-donor gender mismatch was more common in R-PSC than in the nonrecurrent group (P = 0.045). Post-LT malignancies were significantly more common in the nonrecurrent cases compared with all others combined (P = 0.031) and caused deaths in 4. The majority of deaths (11/13) in other groups were due to sepsis complicating graft dysfunction. In conclusion, allograft autoimmune liver disease was seen in 18 (43%) of 42 long-term post-LT PSC patients, with progression in 5 of 18 patients. Features of PSC were seen in 6 (33%) of 18. Native liver XGC negatively impacted post-LT graft and patient survival. Increased incidence of malignancies in the nonrecurrent group may reflect overimmunosuppression in those patients.

Original languageEnglish (US)
Pages (from-to)1127-1136
Number of pages10
JournalHuman Pathology
Volume34
Issue number11
DOIs
StatePublished - Nov 2003
Externally publishedYes

Fingerprint

Sclerosing Cholangitis
Liver Transplantation
Graft Survival
Autoimmune Diseases
Liver Diseases
Tissue Donors
Autoimmune Hepatitis
Liver
Inflammatory Bowel Diseases
Immunosuppression
Allografts
Neoplasms
Sepsis
Histology
Ischemia
Transplants
Recurrence

Keywords

  • Autoimmune
  • Liver transplantation
  • Long-term
  • Sclerosing cholangitis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Khettry, U., Keaveny, A., Goldar-Najafi, A., Lewis, W. D., Pomfret, E. A., Pomposelli, J. J., ... Gordon, F. D. (2003). Liver Transplantation for Primary Sclerosing Cholangitis: A Long-Term Clinicopathologic Study. Human Pathology, 34(11), 1127-1136. https://doi.org/10.1053/j.humpath.2003.07.015

Liver Transplantation for Primary Sclerosing Cholangitis : A Long-Term Clinicopathologic Study. / Khettry, Urmila; Keaveny, Andrew; Goldar-Najafi, Atoussa; Lewis, W. David; Pomfret, Elizabeth A.; Pomposelli, James J.; Jenkins, Roger L.; Gordon, Fredric D.

In: Human Pathology, Vol. 34, No. 11, 11.2003, p. 1127-1136.

Research output: Contribution to journalArticle

Khettry, U, Keaveny, A, Goldar-Najafi, A, Lewis, WD, Pomfret, EA, Pomposelli, JJ, Jenkins, RL & Gordon, FD 2003, 'Liver Transplantation for Primary Sclerosing Cholangitis: A Long-Term Clinicopathologic Study', Human Pathology, vol. 34, no. 11, pp. 1127-1136. https://doi.org/10.1053/j.humpath.2003.07.015
Khettry U, Keaveny A, Goldar-Najafi A, Lewis WD, Pomfret EA, Pomposelli JJ et al. Liver Transplantation for Primary Sclerosing Cholangitis: A Long-Term Clinicopathologic Study. Human Pathology. 2003 Nov;34(11):1127-1136. https://doi.org/10.1053/j.humpath.2003.07.015
Khettry, Urmila ; Keaveny, Andrew ; Goldar-Najafi, Atoussa ; Lewis, W. David ; Pomfret, Elizabeth A. ; Pomposelli, James J. ; Jenkins, Roger L. ; Gordon, Fredric D. / Liver Transplantation for Primary Sclerosing Cholangitis : A Long-Term Clinicopathologic Study. In: Human Pathology. 2003 ; Vol. 34, No. 11. pp. 1127-1136.
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abstract = "The course and outcome of patients after liver transplantation (LT) for primary sclerosing cholangitis (PSC) are still debated. Our purpose is to define retrospectively, the post-LT clinicopathologic findings seen in 51 PSC patients with a follow-up of 2 to 14 years. Of the total 51 patients, 16 with native liver hilar xanthogranulomatous cholangiopathy (XGC) had median graft and patient survival of 573 and 835 days, respectively compared with 2489 and 2794 days, respectively, in 35 patients without XGC. Perioperative complications resulted in 9 early deaths (day 0 to 52). Of the remaining 42 patients, 6 had recurrent PSC (R-PSC) with typical histologic and cholangiographic findings, 12 had autoimmune liver disease-not otherwise specified with histology of autoimmune hepatitis/overlap syndrome, 3 had chronic rejection, 4 had ischemic cholangiopathy, and 17 had no recurrence. The presence of inflammatory bowel disease, total ischemia time of ≥11 hours, recipient-donor ABO and HLA Class I and II matches, and the type of immunosuppression did not affect the post-LT outcome. Recipient-donor gender mismatch was more common in R-PSC than in the nonrecurrent group (P = 0.045). Post-LT malignancies were significantly more common in the nonrecurrent cases compared with all others combined (P = 0.031) and caused deaths in 4. The majority of deaths (11/13) in other groups were due to sepsis complicating graft dysfunction. In conclusion, allograft autoimmune liver disease was seen in 18 (43{\%}) of 42 long-term post-LT PSC patients, with progression in 5 of 18 patients. Features of PSC were seen in 6 (33{\%}) of 18. Native liver XGC negatively impacted post-LT graft and patient survival. Increased incidence of malignancies in the nonrecurrent group may reflect overimmunosuppression in those patients.",
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