The detection of anti-neutrophil cytoplasmic antiodies (ANCA) is now a
routine part of the evaluation of patients clinically suspected of su
ffering from small vessel vasculitis. The factor(s) that trigger the d
evelopment of these autoantibodies and their role in the pathogenesis
of vasculitis is still unclear. We describe four patients who presente
d to us since June 1990. All patients had positive ANCA serology and h
ad clinical evidence of vasculitis. In all patients soon after the est
ablishment of ANCA positivity, a carcinoma of either the respiratory o
r urinary tracts was diagnosed. We suggest that in some cases of ANCA-
associated vasculitis, malignant disease may be a trigger for either t
he generation of these autoantibodies, or the development of vasculiti
s.