Publications about liver transplantation (LTX) for autoimmune hepatitis (AI
H) have started to emerge, but many issues remain unresolved. We reviewed d
ata on 32 patients transplanted for AIH to determine how pretransplantation
and posttransplantation characteristics correlate with recipient outcome,
including disease recurrence. Recipients were 37 +/- 14 years old; 30 of 32
were women. Most had chronic disease (8 +/- 6 years); 25% had fulminant fa
ilure. The majority had ascites (91%), jaundice (88%), elevated prothrombin
time (18 +/- 3 seconds), and hypoalbuminemia (2.7 +/- 0.6 g/dL), All had h
ypergammaglobulinemia (3.0 +/- 1.0 g/dL) and autoantibodies (72% antinuclea
r, 74% smooth muscle). Only one was HLA A1-B8-DR3 positive. Other autoimmun
e disorders affected 25% of patients; half improved after transplantation.
Actuarial survival was 81% at 1 and 2 years posttransplantation. There was
a high frequency of rejection (75% of recipients had 1.7 +/- 0.8 episodes),
and 39% of rejections required OKT3, Among 24 recipients with long-term fo
llow-up (27 +/- 14 months), histologically proven recurrent AIH occurred in
25%, 15 +/- 2 months posttransplantation; half (3 patients) required retra
nsplantation 11 +/- 3 months after diagnosis, After retransplantation 2 of
3 patients had re-recurrence within 3 months; 1 received a third LTx, Recur
rence occurred in 6 of 18 patients transplanted for chronic disease vs. 0 o
f 6 transplanted as fulminants (P = not significant [NS]). Patients with an
d without recurrence had similar rejection profiles. In summary, results of
LTx for AIH are excellent. However, AIH patients have a high frequency of
rejection and often require OKT3. Furthermore, severe recurrent AIH sometim
es develops, particularly in chronic versus fulminant AIH patients and in t
hose already retransplanted for recurrence. Multicenter studies could eluci
date the best posttransplantation immunosuppressive regimens for AIH patien
ts.