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  • Title: Pulmonary giant bulla in Wegener's granulomatosis.
    Author: Mouly S, Brillet G, Stern M, Lesavre P, Guillevin L.
    Journal: Scand J Rheumatol; 2000; 29(5):333-5. PubMed ID: 11093604.
    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.
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