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Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature
  1. B Hervier1,
  2. C Pagnoux2,
  3. C Agard1,
  4. J Haroche3,
  5. Z Amoura3,
  6. L Guillevin2,
  7. M A Hamidou1,
  8. for the French Vasculitis Study Group
  1. 1
    Department of Internal Medicine, CHU Nantes, France
  2. 2
    Department of Internal Medicine, Hôpital Cochin, Paris, France
  3. 3
    Department of Internal Medicine, CHU Pitié-Salpétrière, Paris, France
  1. Dr B Hervier, Department of Internal Medicine, CHU Nantes 44093, France; baptiste.hervier{at}chu-nantes.fr

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.

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Footnotes

  • Competing interests: None.

  • Ethics approval: Ethics committee approval obtained.

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