Antibodies to actin have been proposed as diagnostic markers for type
1 autoimmune hepatitis, Our aims were to determine 1) if testing for a
ntibodies to actin is superior to testing for smooth muscle antibodies
(SMA); 2) if these antibodies identify patients with distinctive clin
ical features; and 3) if the production of antibodies to actin is asso
ciated with genetic risk factors for autoimmune hepatitis, Sera from 9
9 patients with type 1 autoimmune hepatitis were tested, The frequenci
es of HLA B8, DR3, DR4, and A1-B8-DR3 in patient subsets were compared
with those in 80 normal subjects, Seventy-three patients (74%) had an
tibodies to actin, Antibodies to actin were found more commonly in pat
ients with SMA than in patients without them (86% vs, 7%, P < .0001),
Screening only for antibodies to actin and antinuclear antibodies (ANA
) failed to establish the diagnosis of autoimmune hepatitis in 5 patie
nts, Patients with antibodies to actin were younger than seronegative
patients, They were also more commonly DR3-positive than normal subjec
ts and more frequently B8-positive than patients with non-actin-associ
ated SMA (49% vs, 0%, P =.004), Only patients with antibodies to actin
died of liver failure (6% vs, 0%), and 10 of 11 patients requiring li
ver transplantation were seropositive for these antibodies, Indeed, de
ath and liver transplantation occurred more frequently in these patien
ts than in actin-negative patients with ANA (19% vs, 0%, P =.03), We c
onclude that routine screening for antibodies to actin may miss patien
ts with type 1 autoimmune hepatitis, Antibodies to actin are associate
d with HLA B8 and DR3, and they identify patients with a poor prognosi
s.