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Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature.
  1. B Hervier (baptiste.hervier{at}
  1. CHU NANTES, Hotel Dieu, France
    1. C Pagnoux (christian.pagnoux{at}
    1. Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, France
      1. C Agard (christian.agard{at}
      1. CHU NANTES, Hotel Dieu, France
        1. J Haroche (julien.haroche{at}
        1. Service de médecine interne, CHU Pitié-Salpetrière, Paris, France
          1. Z Amoura (zahir.amoura{at}
          1. Service de Médecine Interne, Hopital Pitié-Salpëtrière, Paris, France
            1. L Guillevin (loic.guillevin{at}
            1. Hopital Cochin, France
              1. M A Hamidou (mohamed.hamidou{at}
              1. CHU Hôtel-Dieu. Nantes University Hospital, France


                Objective: Association of pulmonary fibrosis (PF) and anti-neutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis (ANCA-V) is rare. The aim of our study was to describe the clinical presentation of this association.

                Methods: We retrospectively studied 12 patients with ANCA-V associated with “idiopathic” PF.

                Results: There were 3 females and 9 males, and the mean age was 70.7 years. In 8 cases, ANCA-V and PF were diagnosed simultaneously; PF occured earlier in 3 cases, and during the ANCA-V follow-up in one. No patient had argument for intra-alveolar hemorrhage (IAH). ANCA were MPO-ANCAs (myeloperoxydase) in all cases. Seven patients had a blood eosinophilia at diagnosis. Two patients died during the ANCA-V induction therapy. Five patients had a respiratory status worsening and 3 of them died (later than 37 months after diagnosis) from exacerbation of end-stage respiratory failure. The five remaining patients had a respiratory status stability.

                Conclusion: The association of PF and ANCA-V does not seem fortuitous, eventhough their clinical evolutions are clearly not related. PF was the major cause of death. ANCA-V has several characteristics, in accordance with the literature : generally male gender, older age, absence of IAH, frequent blood eosinophilia and ANCAs directed against MPO.

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