Ocular involvement occurs in 40% to 50% of patients with Wegener's granulomatosis. The frequent involvement of the eyes in Wegener's disease is largely based on the anatomical contiguity of the orbit and the upper airways. On the other hand, it is also attributable to an isolated ocular involvement in the form of a focal necrotizing vasculitis. In about 60% of patients suffering from Wegener's granulomatosis there are anticytoplasmic antibodies. There seems to be a correlation between the serum level of ACPA and disease activity. In 10 patients out of 12 with ocular vasculitis due to Wegener's granulomatosis positive ACPA titers were found.