The increased prevalence of primary biliary cirrhosis (PBC) in Sjogren's sy
ndrome and scleroderma has been described. We determined the frequency of a
ntimitochondrial antibodies (AMA) in limited scleroderma, compared with dif
fuse scleroderma, and have considered their clinical significance because t
hey are known to be related to PBC. Outpatients with scleroderma were prosp
ectively tested for the presence of AMA and to determine whether they had e
vidence of liver disease both clinically and by laboratory parameters. Sixt
y-one patients with scleroderma were tested for AMA. Thirty of these had di
ffuse scleroderma. Four patients, all of whom had limited disease (4 of 31
or 13%), had positive AMA. Two had known primary biliary cirrhosis. The oth
er two participants with positive antibodies had no evidence of liver disea
se when examined clinically, but no liver biopsies were performed. They hav
e been followed for >2 years, without clinical evidence of PBC.
Our findings agree with the Literature that shows that 8% to 15% of patient
s with limited scleroderma have AMA. AMA did not occur in diffuse scleroder
ma in our population. Screening for the occurrence of PBC in scleroderma wi
th antimitochondrial antibodies in patients without signs or symptoms of li
ver disease is unnecessary because at this time, we would not give ursodeox
ycholic acid to scleroderma patients with positive AMA and normal alkaline
phosphatases who had no symptoms of PBC.