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Liver transplantation in adults with liver disease due to common variable immunodeficiency leads to early recurrent disease and poor outcome.

Authors
  • Azzu, Vian1
  • Elias, Joshua E1
  • Duckworth, Adam2
  • Davies, Susan2
  • Brais, Rebecca2
  • Kumararatne, Dinakantha S3
  • Gimson, Alexander E S1
  • Griffiths, William J H1
  • 1 The Liver Unit, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom. , (United Kingdom)
  • 2 Department of Histopathology, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom. , (United Kingdom)
  • 3 Department of Immunology, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom. , (United Kingdom)
Type
Published Article
Journal
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
Publication Date
Feb 01, 2018
Volume
24
Issue
2
Pages
171–181
Identifiers
DOI: 10.1002/lt.24979
PMID: 29156507
Source
Medline
License
Unknown

Abstract

Common variable immunodeficiency (CVID) is the most common form of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections, and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features, such as nodular regenerative hyperplasia, inflammation, fibrosis, and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence. There is a statistically significant decrease in 3-year and 5-year survival after liver transplantation in those with CVID-related liver disease (55% at 3 and 5 years) compared with all-comers (89% at 3 years, 81% at 5 years), prompting a need for discussion of suitability of transplantation in this group of patients as well as methods for reducing posttransplantation risk such as scrupulous search for infectious agents and reduction of immunosuppression. Liver Transplantation 24 171-181 2018 AASLD.

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