The incidence, specificity and clinical significance of positivity for seru
m antineutrophil cytoplasmic antibody (ANCA) was investigated Id 60 patient
s with primary Sjogren's syndrome (SjS). The indirect immunofluorescence (I
IF) technique and an enzyme-linked immunosorbent assay (ELISA) were used to
measure ANCA. Purified myeloperoxidase (MPO), lactoferrin (LF), cathepsin-
G (CTG) elastase (HLE) served as ANCA antigens for the ELISA. Ten (16.7%) o
f the 60 SjS patients showed positivity by IIF or perinuclear, but not cyto
plasmic, ANCA. Four of the 60 sera were shown to be positive for LF, four f
or MPO, 0 for CTG and 0 for HLE by ELISA. There was no correlation between
ANCA positivity and clinical features. ANCA in patients with SjS might be a
n epiphenomenon of polyclonal B-cell activation.