We present a case of Wegener's granulomatosis (WG) that responded to antitu
berculous drugs. A 44-year-old woman with multiple nodules on chest radiogr
aph received a diagnosis of pulmonary tuberculosis because open-lung biopsy
specimens showed caseous granulomas. Her chest shadows underwent repeated
resolution after the start of antituberculous treatment, and relapse after
the cessation of the drugs. Antineutrophil cytoplasmic antibody was positiv
e (14 enzyme-linked immunosorbent assay units), and the second lung biopsy
specimens showed necrotizing granulomas and vasculitis without pathogenic o
rganisms. Thus, the patient received a diagnosis of WG and was successfully
treated with trimethoprim/sulfamethoxazole 10 years after her initial eval
uation. Antituberculous drugs were effective in this case of WG.