ANCA-associated lung fibrosis: analysis of 17 patients

Respir Med. 2008 Oct;102(10):1392-8. doi: 10.1016/j.rmed.2008.04.023. Epub 2008 Jul 18.


In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients were past smokers, 3 current smokers and 6 non-smokers. Lung function tests at diagnosis were as follows (% predicted): total lung capacity 73%+/-18, vital capacity 82%+/-23, forced expiratory volume in 1 s (FEV(1)) 88%+/-24, carbon monoxide diffusion capacity of the lung 49%+/-2 (% predicted). Bronchoalveolar lavage results showed an increased cellularity with increased neutrophils counts. High resolution computed tomography of the chest showed prominent fibrosis with some degree of ground-glass attenuation in all patients. These characteristics were similar to the control group. Microscopic polyangiitis (MPA) was a major complicating event in ANCA-positive patients, occurring in 7 patients (anti-myeloperoxidase specificity in 5 patients). Pulmonary fibrosis predated occurrence of MPA in 6 patients and was diagnosed concomitantly with MPA in 1 patient. During the follow-up, 10/17 patients died. The death was directly related to vasculitis in 3 patients. We conclude that patients with pulmonary fibrosis should be evaluated for the presence of ANCA. Patients with positive ANCA testing, particularly if anti-myeloperoxidase, should be carefully monitored to detect the occurrence of microscopic polyangiitis.

MeSH terms

  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Bronchoalveolar Lavage Fluid / immunology
  • Case-Control Studies
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Neutrophils / immunology
  • Peroxidase / immunology
  • Pulmonary Fibrosis / complications
  • Pulmonary Fibrosis / immunology*
  • Pulmonary Fibrosis / mortality
  • Retrospective Studies
  • Smoking
  • Statistics, Nonparametric
  • Survival Rate
  • Vasculitis / complications
  • Vasculitis / immunology*
  • Vasculitis / mortality


  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase