Recurrence of Primary Biliary Cirrhosis after Liver Transplantation

Abstract

Three patients who had undergone orthrotopic liver transplantation for primary biliary cirrhosis and were being maintained on immunosuppressive therapy were investigated 31/2 to 41/2 years later because of the redevelopment of pruritus and mild jaundice. In one patient pigmentation was again evident, and all three had a rise in the titer of serum mitochondrial antibody after an initial fall. Liver histology showed features of primary biliary cirrhosis with non-suppurative destructive cholangitis, lymphoid aggregates, and increased deposition of copper-binding protein in the absence of cholestasis. None of these features was found in patients who had received grafts for other conditions and had lived for comparable periods, nor were they found in patients who had had rejection with bile-duct abnormalities. The overall findings indicate a recurrence of primary biliary cirrhosis in the donor organ.

Pathologic analysis of liver transplantation for primary biliary cirrhosis

“…We can only state that we were unable to diagnose the disease using the criteria offered by Neuberger et a1. (10). The reasons we were unable to diagnose recurrent disease with certainty included the different histopathologic appearance of PBC and chronic rejection, the lack of correlation of AMA levels with liver dysfunction and/or hepatic pathology and our ignorance of the effect of cyclosporin therapy on the histopathology of PBC.…”

“…The difficulty of using pathologic observations for the diagnosis of PBC after transplantation is underscored by the experience at Cambridge (10). Since their original report, the Cambridge group has identified patients who developed a PBC-like syndrome (clinically and pathologically) after transplantation but did not have the disease originally, nor did they convert to AMA positivity (24).…”

“…In addition, because our patients have been maintained on cyclosporin whereas those reported by the Cambridge group having recurrent disease received azathioprine and not cyclosporin, it may be impossible to reconcile the pathologic findings ofthe two groups. Finally, no number of negative cases can prove that PBC or any other disorder cannot recur; only one classic case needs to be documented to prove otherwise (10). Differences in the postoperative course of patients transplanted for PBC have been seen compared to those transplanted for other disorders.…”

“…Neuberger et a1. (10) have suggested that recurrent PBC occurs in patients transplanted for this indication. This conclusion was based on the findings of a consistent hepatic histopathology, the presence of AMA and a posttransplant clinical course consistent with PBC.…”

Liver Transplantation for Primary Biliary Cirrhosis

“…The initial report of recurrence of PBC in three patients by Neuberger et al 15 was not confirmed in the Pittsburgh experience. 16 Recurrence of the disease, using the criteria described by Neuberger et al, 15 was not found by Demetris et al 17 in a recent pathologic review of graft specimens obtained from 106 patients with PBC.…”