Introduction: Pulmonary fibrosis secondary to antineutrophil cytoplasmic antibodypositive vasculitis is rarely reported. Most cases have occurred in association with microscopic polyangiitis.
Case Report: We report the case of an 82-year-old man presenting with pulmonary fibrosis. The CT scan showed honeycombing and traction bronchiectasis with peripheral and basal predominance. The diagnosis of Wegener's granulomatosis was based on the association of positive antineutrophil cytoplasmic antibodies, urinary sediment abnormalities and renal biopsy findings.
Conclusion: We emphasize, through this case, the rarity of interstitial fibrosis as a pulmonary manifestation of vasculitis and we discuss the pathogenesis of this association.
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