Tables
- TABLE 1
Demographics, disease severity, clinical, radiological, functional and microbiological characteristics of the study population
Variables Study population (n=401) Demographics Female sex 317 (79.1%) Age, years 63 (50–71) Body mass index, kg·m−2 21.5 (19.5–24.0) Underweight 58 (14.6%) Former or current smoker 180 (44.9%) Comorbidities GORD 180 (44.9%) Rhinosinusitis 138 (34.4%) Cardiovascular diseases 142 (35.4%) Systemic hypertension 93 (23.2%) Asthma 60 (15.0%) Osteoporosis 69 (17.2%) COPD 32 (8.0%) Depression 34 (8.5%) Anxiety 27 (6.7%) History of neoplastic disease 56 (14.0%) Diabetes 17 (4.2%) BACI score 0 (0–3) Functional evaluation FEV1, % pred 87 (71–101) FEV1 <50% pred 30 (7.9) FVC, % pred, mean±sd 97.5±21.7 Microbiology Chronic infection 120 (35.9%) Chronic Pseudomonas aeruginosa infection 76 (22.7%) Chronic Haemophilus influenzae infection 20 (6.0%) NTM-PD 41 (12.2%) Clinical status Exacerbations 2 (1–3) ≥3 exacerbations in the previous year 132 (32.9%) LTOT 19 (4.7%) Daily sputum 267 (66.6%) Sputum volume, mL 6 (4–20) Chronic treatment Chronic macrolide therapy 40 (10.0%) Chronic antibiotic inhaled therapy 21 (5.2%) Radiology Reiff score 4 (2–6) Number of lobes involved 3 (2–5) Disease severity BSI score 6 (3–9) BSI moderate–severe 244 (63.9%) BSI severe 98 (25.7%) FACED score 2 (1–3) FACED moderate–severe 141 (36.0%) Data are presented as median (interquartile range) unless otherwise stated. GORD: gastro-oesophageal reflux disease; COPD: chronic obstructive pulmonary disease; BACI: before, after, control, impact; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; NTM-PD: nontuberculous mycobacterial pulmonary disease; LTOT: long-term oxygen therapy; BSI: bronchiectasis severity index; FACED: FEV1, age, chronic colonisation, extension, dyspnoea.
- TABLE 2
Prevalence of bronchiectasis adults (n=401) with any, primary, secondary or treatable immunodeficiencies according to five different sets of immunological tests (S1–S5)
S1 S2 S3 S4 S5 Any immunodeficiency 36 (8.9%) 93 (23.2%) 179 (44.6%) 179 (44.6%) 179 (44.6%) Primary immunodeficiency 29 (7.2%) 83 (20.7%) 158 (39.4%) 158 (39.4%) 158 (39.4%) Isolated IgG subclass deficiency 0 0 36 (9%) 36 (9%) 36 (9%) Isolated IgG1 subclasses deficiency
0 0 3 (0.8%) 3 (0.8%) 3 (0.8%) Isolated IgG2 subclasses deficiency
0 0 2 (0.5%) 2 (0.5%) 2 (0.5%) Isolated IgG3 subclasses deficiency
0 0 6 (1.5%) 6 (1.5%) 6 (1.5%) Isolated IgG4 subclasses deficiency
0 0 24 (5.9%) 24 (5.9%) 24 (5.9%) IgG1 and IgG3 subclasses deficiency
0 0 1 (0.2%) 1 (0.2%) 1 (0.2%) Isolated IgM deficiency 0 0 3 (0.7%) 3 (0.7%) 3 (0.7%) Unclassified antibody deficiency 0 0 6 (1.5%) 6 (1.5%) 6 (1.5%) CVID 0 0 2 (0.5%) 2 (0.5%) 2 (0.5%) Severe combined immunodeficiency 0 0 0 0 0 Combined immunodeficiency 0 0 0 0 0 Hyper-IgE syndrome 0 0 0 0 0 Isolated IgA deficiency 2 (0.5%) 2 (0.5%) 2 (0.5%) 2 (0.5%) 2 (0.5%) DiGeorge Syndrome 1 (0.2%) 1 (0.2%) 1 (0.2%) 1 (0.2%) 1 (0.2%) Unclassified immunodeficiency 26 (6.5%) 80 (20%) 108 (26.9%) 108 (26.9%) 108 (26.9%) Secondary immunodeficiency 7 (1.7%) 10 (2.5%) 21 (5.2%) 21 (5.2%) 21 (5.2%) Immunosuppressive drugs 1 (0.2%) 3 (0.7%) 11 (2.7%) 11 (2.7%) 11 (2.7%) Steroids
1 (0.2%) 3 (0.7%) 8 (2%) 8 (2%) 8 (2%) Biologics
0 (0%) 0 (0%) 2 (0.5%) 2 (0.5%) 2 (0.5%) Antiproliferative agents
0 (0%) 0 (0%) 1 (0.2%) 1 (0.2%) 1 (0.2%) Haematological malignancy 6 (1.5%) 6 (1.5%) 7 (1.7%) 7 (1.7%) 7 (1.7%) Transplant 0 1 (0.2%) 3 (0.7%) 3 (0.7%) 3 (0.7%) Other aetiologies 113 (28.2%) 113 (28.2%) 67 (16.7%) 67 (16.7%) 67 (16.7%)# Idiopathic bronchiectasis 252 (62.8%) 195 (48.6%) 155 (38.7%) 155 (38.7%) 155 (38.7%) Treatable immunodeficiencies 15 (3.7%) 37 (9.2%) 67 (16.7%) 67 (16.7%) 67 (16.7%) S1: complete blood count, and total IgG, IgA and IgM serum levels; S2: S1 plus IgG subclasses; S3: S2 plus lymphocyte subsets; S4: S3 plus total IgE; S5: S4 plus HIV testing. CVID: common variable immunodeficiency. #: including 26 post-infective, eight primary ciliary dyskinesia and five allergic bronchopulmonary aspergillosis.
- TABLE 3
Demographic, clinical, functional, radiological, and microbiological characteristics of the three study groups: patients with primary immunodeficiency (Group A), patients with secondary immunodeficiency (Group B) and patients with idiopathic bronchiectasis (Group C)
Variables Group A (n=158) Group B (n=21) Group C (n=155) p-value Demographics Female sex 122 (77.2%) 13 (61.9%) 129 (83.2%) 0.06 Age, years 65 (52–73) 70 (65–73) 62 (48–70) 0.009# Body mass index, kg·m−2 21.1 (19.2–24.0) 23.5 (21.9–25.5) 21.4 (19.7–24.0) 0.08 Underweight 27 (17.2%) 2 (10.0%) 20 (13.0%) 0.48 Former or current smoker 63 (39.9%) 13 (61.9%) 77 (49.7%) 0.07 Comorbidities GORD 71 (44.9%) 11 (52.4%) 74 (47.7%) 0.76 Rhinosinusitis 58 (36.7%) 7 (33.3%) 45 (29.0%) 0.35 Cardiovascular diseases 66 (41.8%) 12 (57.1%) 43 (27.7%) 0.004¶ Systemic hypertension 43 (27.2%) 8 (38.1%) 29 (18.7%) 0.06 Asthma 24 (15.2%) 1 (4.8%) 29 (18.7%) 0.25 Osteoporosis 24 (15.2%) 10 (47.6%) 26 (16.8%) 0.001+ COPD 16 (10.1%) 2 (9.5%) 9 (5.8%) 0.32 Depression 17 (10.8%) 3 (14.3%) 10 (6.5%) 0.24 Anxiety 11 (7.0%) 2 (9.5%) 11 (7.1%) 0.84 History of neoplastic disease 23 (14.6%) 12 (57.1%) 13 (8.4%) <0.0001§ Diabetes 6 (3.8%) 7 (33.3%) 3 (1.9%) <0.0001ƒ Functional FEV1, % pred 85.9±24.9 84.6±27.2 85.6±20.8 0.97 FEV1 <50% pred 13 (8.8%) 2 (10.0%) 9 (6.2%) 0.62 FVC, % pred 98.5±23.2 97.5±25.1 97.3±18.6 0.89 Microbiological Chronic infection 49 (36.6%) 5 (29.4%) 46 (35.4%) 0.84 Chronic Pseudomonas aeruginosa infection 31 (23.1%) 3 (17.7%) 30 (23.1%) 0.95 Chronic Haemophilus influenzae infection 7 (5.3%) 3 (17.7%) 8 (6.2%) 0.14 NTM-PD 18 (13.4%) 3 (17.7%) 11 (8.5%) 0.29 Clinical Exacerbations 2 (1–3) 1 (0–3) 2 (1–3) 0.97 ≥3 exacerbations in the previous year 51 (32.3%) 8 (38.1%) 49 (31.6%) 0.84 LTOT 11 (7.0%) 2 (9.5%) 4 (2.6%) 0.09 Daily sputum 104 (65.8%) 15 (71.4%) 107 (69.0%) 0.77 Sputum volume, mL 7 (4.5–20.0) 5.5 (3–50) 6 (5–20) 0.93 Chronic macrolide therapy 16 (10.1) 1 (4.8) 11 (7.1) 0.62 Chronic inhaled antibiotic therapy 8 (5.1) 1 (4.8) 10 (6.5) 0.86 Radiological Reiff score 4 (2–6) 4 (2–6) 4 (3–6) 0.99 Number of lobes involved 3 (2–4) 4 (2–6) 4 (2–5) 0.59 Disease severity BSI score 6 (4–9) 6 (3.0–8.5) 6 (3–8) 0.11 FACED score 2 (1–3) 2 (2–3) 2 (1–3) 0.06 BACI score 0 (0–3) 6 (3–10) 0 (0–3) 0.0001## Data are presented as median (interquartile range) or mean±sd, unless otherwise stated. GORD: gastro-oesophageal reflux disease; COPD: chronic obstructive pulmonary disease; FEV1, forced expiratory volume in 1 s; FVC: forced vital capacity; NTM-PD: nontuberculous mycobacterial pulmonary disease; LTOT: long-term oxygen therapy; BSI: bronchiectasis severity index; FACED: FEV1, age, chronic colonisation, extension, dyspnoea; BACI: before, after, control, impact. #; Group B versus Group C, p=0.008. ¶: Group A versus Group C, p=0.009; Group B versus Group C, p=0.006. +: Group A versus Group B, p=0.0004; Group B versus Group C, p=0.001. §: Group A versus Group C, p<0.0001; Group B versus Group C, p<0.0001. ƒ: Group A versus Group C, p<0.0001; Group B versus Group C, p<0.0001. ##: Group A versus Group B, p<0.0001; Group B versus Group C, p<0.0001.
