Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature

Ann Rheum Dis. 2009 Mar;68(3):404-7. doi: 10.1136/ard.2008.096131. Epub 2008 Oct 28.


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.

Publication types

  • Review

MeSH terms

  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / immunology
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Prognosis
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / immunology
  • Retrospective Studies
  • Vasculitis / complications*
  • Vasculitis / immunology


  • Antibodies, Antineutrophil Cytoplasmic