Background/Aims: Antineutrophil cytoplasmic antibodies have been repor
ted to occur more frequently in healthy first-degree relatives of pati
ents with ulcerative colitis than in healthy controls. The aim of this
study was to determine their prevalence in families in which more tha
n one member was affected with inflammatory bowel disease. Methods: Wi
th use of an indirect immunofluorescence method, 168 affected members
and 197 unaffected first-degree relatives in 56 such families were stu
died, Results: Antibodies were detected in 46% of patients with ulcera
tive colitis without a positive family history and in 44% of those wit
hin families with several affected members, There was no evidence of c
lustering of antibodies in particular families. Within families in whi
ch both ulcerative colitis and Crohn's disease coexisted, antibodies w
ere detected primarily in patients with ulcerative colitis. Antibodies
were seldom present (3%) in those patients with Crohn's disease, whet
her they were familial or nonfamilial cases. Similarly, antibodies wer
e seldom present (3%) in controls or the unaffected first-degree relat
ives of patients with inflammatory bowel disease. Conclusions: Antineu
trophil cytoplasmic antibodies ave associated with ulcerative colitis,
and their presence is not increased in the first-degree relatives of
patients with ulcerative colitis. They are move likely to be a consequ
ence of the disease than a subclinical genetic disease marker.