Recurrence of primary biliary cirrhosis after living related donor liver transplantation

Abstract

Primary biliary cirrhosis (PBC) is an intractable and progressive cholestatic liver disease for which liver transplantation (LT) is one of the best treatment options. It is well known that PBC frequently recur after LT, although, there is still some controversy about the factors causing PBC recurrence. The purpose of this study was to elucidate whether PBC recurs after both cadaveric and living related-donor (LRD) LT in Japanese recipients. (Materials and Methods) By the middle of August, 1997, 60 adult patients had received liver grafts from living related adult donors at 13 different institutes in Japan. Among them, 10 patients with PBC who had undergone LRDLT and 9 who had undergone cadaveric LT abroad were investigated. These PBC recipients were followed up at different institutes for more than 1 year after both LRDLT and cadaveric LT, with maximum periods of 4 years and 9 years, respectively. The major immunosuppressant used was cyclosporin A or FK506 with corticosteroids. (Results) Seven of 10 patients in the LRDLT group were positive for anti-mitochondrial antibody (AMA) with a high titer, 6 of 9 showed re-elevation of both ALP and IgM, and 2 of 5 showed destructive cholangitis as well as chronic non-suppurative destructive cholangititis, whereas only one of 9 patients in the cadaveric LT group revealed elevation of ALP and 2 were positive for AMA. Analysis of HLA typing indicated that 8 cases of LRDLT had one or two matching loci in class II (DR) and one matching locus in class I (A and B), although there were no specific loci or DNA typing characteristics in these groups. Only two of 5 cadaveric LT recipients with abnormalities of ALP and a positive AMA titer had a matching class II (DR) locus. (Conclusion) Serological markers of PBC show a much higher tendency to become positive after LRDLT than after cadaveric LT in Japanese patients. The reason for this high incidence of recurrence after LRDLT is still unclear, although, HLA matching might play an important role. All the LRDLT patients showed HLA matching, especially at the DR locus, because of the fact that close relatives were used as donors. Quality of life was excellent after both LRDLT and cadaveric LT, although in the long term after LT, the possible recurrence of PBC needs to be considered.