Pulmonary giant bulla in Wegener's granulomatosis

The association of lung emphysema with severe systemic antineutrophil cytop lasm antibodies (ANCA)-positive vasculitis, such as Wegener's granulomatosi s is unusual since only four cases have been described previously. We repor t the first case of a 30 year-old smoker man presenting with biopsy-proven Wegener's granulomatosis, who developed a bullous emphysema during severe a ctive lung vasculitis, in association with positive ANCA disclosing an anti -myeloperoxydase pattern. Alpha I-antitrypsin deficiency, a known risk fact or of lung emphysema recently found to be associated with anti-proteinase 3 -positive vasculitis, was not present in this patient. Cigarette smoking, i n association with severe lung vasculitis, might have contributed to the de velopment of this emphysematous lesion.