Anti-CD25 and Tacrolimus Therapy May Not Prevent Early Primary
Biliary Cirrhosis Recurrence After Liver Transplantation: Two
Case Reports
B. Foroncewicz, K. Mucha, L. Paczek, U. Oldakowska-Jedynak, B. Go ´ rnicka, K. Zieniewicz,
P. Nyckowski, and M. Krawczyk
ABSTRACT
Primary biliary cirrhosis (PBC) is an immune-mediated disorder of unknown cause
characterized by progressive destruction of intrahepatic bile ducts and the presence of
antimitochondrial antibodies. There is no known cure for PBC, and treatment generally
includes various combinations of ursodeoxycholic acid and immunosuppressive agents.
However, in most patients with end-stage PBC, liver transplantation offers a good quality
of life.
Recurrent PBC after transplantation is controversial, because most patients with suspected
recurrent disease are asymptomatic. Antimitochondrial antibodies frequently persist and
do not correlate with disease recurrence. However, most studies support disease recur-
rence within the graft. The effects of immunosuppression may modify or delay disease
expression within the graft. If PBC recurs, intermediate-term patient and graft survivals
are excellent, but the long-term outcome remains unknown. Many immunosuppressive
agents have been studied with regard to their anti-recurrence properties; however, no
standard therapy has been established for this group of patients. In this study we present
two patients transplanted for PBC who displayed early recurrence of disease confirmed by
liver biopsy and elevated serum AMA. Both individuals received the same immunosup-
pressive regimen. The data suggest that two doses of daclizumab and tacrolimus
monotherapy in the early posttransplant period is insufficient to prevent recurrence of
PBC. Addition of glucocorticoids may have beneficial effects in these patients.
INTRODUCTION
P
RIMARY BILIARY cirrhosis (PBC) is an immune-
mediated disorder of unknown etiology characterized
by progressive destruction of intrahepatic bile ducts and the
presence of antimitochondrial antibodies (AMA). The dis-
ease, which has a strong female preponderance (10:1), is
commonly associated with other autoimmune disorders
including sicca complex, CREST syndrome, rheumatoid
arthritis, thyroiditis, pernicious anemia, and renal tubular
acidosis.
1
In approximately 90% of patients with PBC the
serum contains antibodies against a lipoprotein component
of the inner mitochondrial membrane.
2
Several agents have been used to treat patients with
confirmed PBC. Ursodeoxycholic acid (UDCA), a hydro-
philic bile acid seems to slow the progression of disease,
possibly by reducing the concentration of toxic bile acids in
the hepatic pool. However, no antifibrotic effect of the
UDCA has been observed. Cyclosporine (CsA) had shown
some early promise, but its long-term use has yielded only
moderate benefit compared with the side effects.
3
Other
immunosuppressive agents including azathioprine, metho-
trexate, chlorambucil, and prednisone have met with mod-
est success. Interferon-gamma may have immunopatho-
genic importance in PBC. However, neither UDCA nor
From the Department of Immunology, Transplant Medicine,
and Internal Diseases (B.F., K.M., L.P.), Transplantation Institute,
the Department of Pathology (B.G.), and the Department of
General, Transplantation, and Liver Surgery (K.Z., P.N., M.K.),
Medical University of Warsaw, Warsaw, Poland.
Address reprint requests to B. Foroncewicz, Department of
Immunology, Transplant Medicine, and Internal Diseases, Med-
ical University of Warsaw, Nowogrodzka 59 Str., PL02-006
Warsaw, Poland.
0041-1345/03/$–see front matter © 2003 by Elsevier Inc. All rights reserved.
doi:10.1016/S0041-1345(03)00835-2 360 Park Avenue South, New York, NY 10010-1710
2310 Transplantation Proceedings, 35, 2310 –2312 (2003)