Serum concentrations of the various complement components including th
e classical and the alternative pathways were determined in 58 control
healthy subjects and 80 patients with systemic sclerosis (SSC). The m
ean concentrations of Clq, C2, C5, C6, C7, C9, and factor B were signi
ficantly increased in the SSC patients in comparison to controls, whil
e the increases were not significant for C3 and C8. C4 was an exceptio
n in that the mean levels were found to be decreased, with 18 patients
having levels <65% of the mean normal value. Properdin was also found
to be decreased, but not significantly. We found a similarity between
the pattern of serum complement component concentrations in SSC patie
nts and patients with primary biliary cirrhosis, two disorders frequen
tly associated in the same patients. The significance of complement co
mponent patterns in these diseases is discussed.