title: Pulmonary giant bulla in Wegener's granulomatosis.
abstract: The association of lung emphysema with severe systemic antineutrophil cytoplasm antibodies (ANCA)-positive vasculitis, such as Wegener's granulomatosis is unusual since only four cases have been described previously. We report the first case of a 30 year-old smoker man presenting with biopsy-proven Wegener's granulomatosis, who developed a bullous emphysema during severe active lung vasculitis, in association with positive ANCA disclosing an anti-myeloperoxydase pattern. Alpha 1-antitrypsin deficiency, a known risk factor of lung emphysema recently found to be associated with anti-proteinase 3-positive vasculitis, was not present in this patient. Cigarette smoking, in association with severe lung vasculitis, might have contributed to the development of this emphysematous lesion.
authors.affiliation: Department of Nephrology, Necker Hospital, Paris, France. smouly@email.unc.edu
authors.forename: S
authors.initials: S
authors.lastname: Mouly
authors.forename: G
authors.initials: G
authors.lastname: Brillet
authors.forename: M
authors.initials: M
authors.lastname: Stern
authors.forename: P
authors.initials: P
authors.lastname: Lesavre
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D009195:Peroxidase / Q000276:immunology; D011656:Pulmonary Emphysema / Q000097:blood / Q000209:etiology* / Q000473:pathology; D012907:Smoking
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase