TABLE 1

Univariate analysis of the association between baseline characteristics and massive haemoptysis (MH) occurrence in patients with cystic fibrosis

CharacteristicPatients without MHPatients with MHHazard ratio (95% CI)p-value
Subjects n4715
Age years median (IQR)22.0 (20.5–30.0)19.0 (16.0–22.0)1.01 (0.96–1.04)0.93
Male28 (59.6%)6 (40.0%)0.97 (0.33–2.85)0.96
Homozygous ΔF508/ΔF50818 (38.3%)6 (40.0%)1.04 (0.36–3.01)0.94
Heterozygous ΔF50818 (38.3%)5 (33.3%)1.62 (0.53–4.97)0.40
Body mass index kg·m−220.1±2.218.5 ±2.750.79 (0.61– 1.02)0.075
Severe malnutrition8 (17.0%)7 (46.7%)2.69 (0.94–7.68)0.064
FEV1 % predicted57.3±24.671.9 ±19.81.01 (0.98–1.03)0.55
FVC % predicted74.8±20.679.7 ±19.60.99 (0.96–1.03)0.69
EPI41 (87.2%)12 (80.0%)1.12 (0.25–5.04)0.88
ABPA12 (25.5%)9 (60.0%)6.03 (1.97–18.45)0.002
Liver cirrhosis1 (2.1%)4 (25.0%)ND
Osteoporosis4 (8.5%)1 (6.7%)ND
Diabetes1(2.1%)5 (33.3%)5.66 (1.84–17.42)0.002
Thrombocytopenia1 (2.1%)3 (20.0%)ND
Bacteriology
 Pseudomonas aeruginosa34 (72.3%)8 (53.3%)0.46 (0.15–1.38)0.17
 MSSA22 (46.8%)8 (53.3%)1.53 (0.53–4.42)0.43
 MRSA9 (19.1%)1 (6.7%)1.17 (0.14–9.41)0.88
 Aspergillus10 (21.3%)2 (13.3%)0.88 (0.20–3.96)0.87
 Stenotrophomonas3 (6.4%)1 (6.7%)ND
 Achromobacter2 (4.3%)1 (6.7%)ND
Long-term treatment
 RhDNase37 (78.7%)13 (86.7%)2.19 (0.28–16.85)0.45
 Inhaled tobramycin9 (19.1%)3 (20.0%)1.26 (0.34–4.71)0.73
 Inhaled colimycin12 (25.5%)4 (25.0%)1.05 (0.33–3.36)0.93
 Triazole9 (19.1%)6 (40.0%)4.13 (1.41–12.07)0.009
 >4 i.v. antibiotic courses11 (23.9%)3 (20.0%)1.08 (0.30–3.90)0.90
 Exacerbations per year n (range)2 (0–3)1 (0–3)1.07 (0.85–1.35)0.57

Data are presented as mean±sd or n (%), unless otherwise stated. Hazard ratios were calculated using Cox regression models. Multivariate analysis was not performed due to the limited sample size and MH event number. Bold font indicates statistically significant p-values. Genotype was known for 61 out of the 62 patients. IQR: interquartile range; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; EPI: exocrine pancreatic insufficiency; ABPA: allergic bronchopulmonary aspergillosis; MSSA: methicillin-sensitive Staphylococcus aureus; MRSA: methicillin-resistant S.aureus; RhDNase: recombinant human deoxyribonuclease (dornase alfa); ND: not done.