Outcomes of orthotopic liver transplantation for hepatic sarcoidosis: An analysis of the United Network for organ sharing/organ Procurement and Transplantation Network data files for a comparative study with cholestatic liver diseases

Abstract

Hepatic sarcoidosis is a rare indication for liver transplantation. Using the United Network for Organ Sharing (UNOS)/Organ Procurement and Transplantation Network (OPTN) database, we evaluated patient and graft survival after orthotopic liver transplantation for sarcoidosis between October 1987 and December 2007. We assessed the potential prognostic value of multiple demographic and clinical variables, and we also compared these patients to a case-matched group of patients with primary sclerosing cholangitis (PSC) or primary biliary cirrhosis (PBC). The 1-and 5-year survival rates for the sarcoidosis group were 78% and 61%, respectively, and these rates were significantly worse than the rates for the PSC/PBC group (P ¼ 0.001). Disease recurrence in the liver is a rare cause of graft loss or patient death. Three deaths occurred in the sarcoidosis group because of recurrent hepatic sarcoidosis, and 1 death was a result of cardiac sarcoidosis. A univariate analysis identified an increasing donor risk index as a significant negative factor for outcomes for the sarcoidosis group [hazard ratio (HR) ¼ 2.06, confidence interval (CI) ¼ 1.04-4.06, P ¼ 0.037], but this finding was not found in a multivariate analysis, in which no independent predictors were found to have a significant impact. A case-matched univariate analysis demonstrated that sarcoidosis and morbid obesity were significant negative factors for outcomes, and in a multivariate analysis, sarcoidosis continued to predict worse outcomes (HR ¼ 2.39, CI ¼ 1.21-4.73, P ¼ 0.012). In conclusion, an analysis of the UNOS/OPTN database indicates that the patient and allograft survival rates for hepatic sarcoidosis are satisfactory, but they are worse in comparison with the rates for other cholestatic liver diseases.

Long-Term Outcomes of Liver Transplantation for Hepatic Sarcoidosis: A Single Center Experience

“…This adds to our recent publication on this subject when we found statistically worse outcomes (both graft and patient survival) for sarcoidosis patients when compared with other cholestatic diseases like PSC and PBC. 25 Previous studies, however, showed similar outcomes for these patient groups but these were small and selection bias remains a concern. 20,21 One possible reason for relatively poorer outcome of liver transplantation for these patients could be that sarcoidosis is a multi-organ disease with special predilection for vital organs.…”

Etiologies of Sarcoidosis

“…Most commonly, sarcoidosis is discovered in donor solid organ allografts in recipients with a history of sarcoidosis [73][74][75][76][77][78][79][80], presumably inducing a granulomatous response by the recipient's immune system [81]. One report of sarcoidosis presenting following bone marrow transplantation suggests that this susceptibility may depend on donor MHC haplotypes [82].…”

Atteinte hépatosplénique symptomatique révélatrice d’une sarcoïdose systémique

“…Les résultats de la transplantation hépatique au cours de la sarcoïdose sont satisfaisants mais sont moins bons qu'au cours des autres hépatopathies chroniques cholestatiques [32]. De même, les récidives sur greffon sont possibles et seront les principales causes de rejet et de décès chez les greffés [31,32].…”

“…De même, les récidives sur greffon sont possibles et seront les principales causes de rejet et de décès chez les greffés [31,32]. La splénectomie peut être aussi envisagée dans les atteintes spléniques réfractaires et/ou compliquées de la sarcoïdose [19].…”