A 53-year-old man was admitted to our hospital with a skin eruption, a high fever, and diplopia in April of 1992. He had been given a diagnosis of diffuse panbronchiolitis in 1981. After administration of erythromycin began in 1987, symptoms and chest roentgenographic findings gradually improved. Arteritis of peribronchial muscular arteries with medial destruction was seen in lung biopsy specimens, and periglomerular granulomatous inflammation and necrotizing glomerulitis were seen in renal biopsy specimens. P-ANCA was found, but a test for cytoplasmic ANCA (which is the most specific antigen of Wegener's granulomatosis) was negative. Chronic pulmonary infection due to diffuse panbronchiolitis might have formed ANCA, which may have caused the vasculitis in this patient.