PT  - JOURNAL ARTICLE
AU  - Hervier, B
AU  - Pagnoux, C
AU  - Agard, C
AU  - Haroche, J
AU  - Amoura, Z
AU  - Guillevin, L
AU  - Hamidou, M A
AU  - for the French Vasculitis Study Group
TI  - Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature
AID  - 10.1136/ard.2008.096131
DP  - 2009 Mar 01
TA  - Annals of the Rheumatic Diseases
PG  - 404--407
VI  - 68
IP  - 3
4099  - http://ard.bmj.com/content/68/3/404.short
4100  - http://ard.bmj.com/content/68/3/404.full
SO  - Ann Rheum Dis2009 Mar 01; 68
AB  - 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.