Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature. Issue 3 (28th October 2008)

• Record Type: Journal Article
• Authors: Hervier, B; Pagnoux, C; Agard, C; Haroche, J; Amoura, Z; Guillevin, L; Hamidou, M A
• Is Part Of: Annals of the rheumatic diseases. Volume 68:Issue 3(2009)
• Page Start: 404
• Page End: 407
• Publication Date: 2008-10-28
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2008.096131
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objective: To describe the clinical presentation of the association between pulmonary fibrosis (PF) and systemic vasculitis related to antineutrophil cytoplasmic antibodies (ANCA-V). Methods: 12 patients (three female, mean age 70.7 years) with ANCA-V associated with "idiopathic" PF were studied retrospectively. Results: ANCA-V and PF were diagnosed simultaneously in eight cases; PF occurred earlier in three cases and during ANCA-V follow-up in one. No patient had intra-alveolar haemorrhage (IAH). ANCA were myeloperoxidase (MPO)-ANCA in all cases. Seven patients had blood eosinophilia at diagnosis. Two patients died during ANCA-V induction therapy. The respiratory status of five patients worsened and three of them died from exacerbation of end-stage respiratory failure. The five remaining patients had a stable respiratory status. Conclusion: The association of PF and ANCA-V does not seem to be fortuitous, even though their clinical evolutions are clearly not related. PF was the major cause of death.