Table 1 Clinical features of the patient groups at the time of first bronchoscopy
AcuteRelapseRemissionNormalIPF
Patient numbers111210822
Sex: male675316
Age (mean)5259504570
Smoking: never/ex/current5/6/04/7/13/7/02/5/15/12/3
Pack years (mean)1919211330
FVC (mean, SE)108 (3.7)109 (4.3)112 (7.2)N/A73 (6.6)
TLCO (mean, SE)90 (4.7)87 (4.6)88 (4.7)N/A49 (3.4)
CRP (mean, SE)23 (6.0)45 (26.0)5.8 (5.3)<16 (10.3)
ANCA positivity100%71%33%0%0%
PR3 positivity73%50%17%0%0%
MPO positivity18%14%0%0%0%
BVAS (mean, SE)5.4 (1.4)5.6 (1.6)0.5 (0.3)0%0%
VDI (mean, SE)3.2 (0.7)4.3 (0.6)2 (0.4)0%0%
Bronchiectasis18%43%50%N/A74%*
Fibrosis73%36%33%N/A100%
Cavities18%50%17%N/A0
Nodules91%71%66%N/A32%
Emphysema18%18%22%N/A32%
Haemoptysis9%14%0%N/A0%
  • Characteristics relate to the time of patients’ first bronchoscopy. Data are presented as the mean (SE).

  • Normal C-reactive protein (CRP) at our institution is <10.

  • In Wegener granulomatosis, pulmonary fibrosis was limited focal disease in all but one case.

  • ANCA, antineutrophil cytoplasmic antibody; BVAS, Birmingham Vasculitis Activity Score; FVC, forced vital capacity (% predicted); IPF, idiopathic pulmonary fibrosis; MPO, myeloperoxidase antibody (serum); N/A, not applicable; PR-3, proteinase-3 antibody (serum); TLCO, total lung diffusing capacity for carbon monoxide (% predicted); VDI, vasculitis damage index.

  • *Bronchiectasis in patients with IPF describes traction bronchiectasis.