The aim of study was to analyse ventilation and perfusion (V/Q) lung scan findings in a series of Italian patients with Takayasu's arteritis. Eighteen consecutive patients underwent V/Q lung planar scintigraphy and single-photon emission tomography (SPET). Before perfusion scan acquisition was started, a first-pass study with (99m)Tc-macroaggregates of albumin was performed to assess the right ventricular ejection fraction (RVEF). All patients had normal chest X-rays and were symptom free at the time of the investigation. They also underwent echocardiography to evaluate pulmonary artery pressure and in 13 patients respiratory function tests were performed. In four patients, perfusion lung scan was repeated after 1 year. In 10/18 patients (55.5%), 43 unmatched lobar, segmental or subsegmental perfusion defects were found on planar images; ventilation scintigraphy was normal in all cases. On SPET images, 55 defects were found; no defects were found with SPET in the remaining patients who had normal planar images. All patients had normal RVEF and 5/13 patients had mild restrictive-obstructive lung disease. The pulmonary artery pressure was increased in two patients with perfusion defects. In the four patients who had repeat scintigraphy, all defects remained unchanged. The prevalence of lung perfusion abnormalities observed in Italian patients with Takayasu's arteritis is within the range of values reported in other countries, and V/Q planar scintigraphy is sufficient for the screening of patients.