Acute | Relapse | Remission | Normal | IPF | |
Patient numbers | 11 | 12 | 10 | 8 | 22 |
Sex: male | 6 | 7 | 5 | 3 | 16 |
Age (mean) | 52 | 59 | 50 | 45 | 70 |
Smoking: never/ex/current | 5/6/0 | 4/7/1 | 3/7/0 | 2/5/1 | 5/12/3 |
Pack years (mean) | 19 | 19 | 21 | 13 | 30 |
FVC (mean, SE) | 108 (3.7) | 109 (4.3) | 112 (7.2) | N/A | 73 (6.6) |
TLCO (mean, SE) | 90 (4.7) | 87 (4.6) | 88 (4.7) | N/A | 49 (3.4) |
CRP (mean, SE) | 23 (6.0) | 45 (26.0) | 5.8 (5.3) | <1 | 6 (10.3) |
ANCA positivity | 100% | 71% | 33% | 0% | 0% |
PR3 positivity | 73% | 50% | 17% | 0% | 0% |
MPO positivity | 18% | 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% |
Bronchiectasis | 18% | 43% | 50% | N/A | 74%* |
Fibrosis | 73% | 36% | 33% | N/A | 100% |
Cavities | 18% | 50% | 17% | N/A | 0 |
Nodules | 91% | 71% | 66% | N/A | 32% |
Emphysema | 18% | 18% | 22% | N/A | 32% |
Haemoptysis | 9% | 14% | 0% | N/A | 0% |
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.