Wegener's granulomatosis is a systemic vasculitis characterized by nec
rotizing granulomata and without treatment is associated with a poor p
rognosis. Antineutrophil cytoplasmic antibodies (ANCA) have proved to
be a useful serological marker allowing for early diagnosis and treatm
ent of this condition. We report two cases of patients who were ANCA-n
egative on presentation despite clinical suspicion of Wegener's granul
omatosis. In both cases the patients developed a positive ANCA titre a
s the disease progressed.