Figures
- Fig. 1—
Influence of the presence of a) bactericidal/permeability-increasing protein-antineutrophil cytoplasmic antibodies (BPI-ANCA) and b) immunoglobulin A-anti-Saccharomyces cerevisiae antibodies (IgA-ASCA) on progression-free survival in the 144 cystic fibrosis patients. –––––: BPI-ANCA not present; - - - - -: BPI-ANCA present; ········: IgA-ASCA not present; – – · – – ·: IgA-ASCA present. a) p = 0.05, b) p = 0.045.
Tables
- Table 1—
Initial clinical features and cystic fibrosis(CF) genotype of the study patients
Subjects n 144 Age yrs 25±5.9 (17–43) Sex ratio 1.03 Male 73 (50.7) Female 71 (49.3) Past medical history of autoimmune disease 4 (2.8) Past medical history of drug hypersensitivity 50 (34.7) CFTR gene mutation F508del/F508del 57 (39.6) Others 87 (60.4) Pulmonary involvement 143 (99.3) Chronic exocrine pancreatic insufficiency 134 (93.1) Chronic sinusitis 78 (54.2) BMI ≤19 kg·m−2 67 (46.5) BMI kg·m−2 19.57±2.8 Chronic diarrhoea 51 (35.4) CF-related liver disease 46 (31.9) Diabetes mellitus 34 (23.6) Arthralgia 13 (9) Associated with polyarthritis 2 (1.3) Associated with oligo/monarthritis 3 (2.1) FEV1 % pred 52.2±21.9 FEV1 >75% pred 23 (16) 75%≥FEV1≥30% pred 90 (65.2) FEV1 <30% pred 31 (21.5) Reversible airway obstruction with β2-agonists 37 (25.7) Antibiotic courses for pulmonary exacerbation in past 12 months# 117 (81.3) 0 27 (18.7) 1 35 (24.3) 2 26 (18.1) 3 36 (25) ≥4 20 (13.9) Data are presented as mean±sd (range), n (%) or mean±sd, unless otherwise stated. CFTR: CF transmembrane conductance regulator gene; BMI: body mass index; FEV1: forced expiratory volume in 1 s; % pred: % predicted. #: mean number of courses = 1.6.
- Table 2—
Initial laboratory findings in the 144 cystic fibrosis patients
Microbiology P. aeruginosa colonisation 113/144 (78.5) Multidrug-resistant Pseudomonas 66/136 (48.5) Pan-resistant Pseudomonas 8/136 (5.9) S. aureus colonisation 117/144 (82.5) Methicillin-resistant S. aureus 22/109 (20.2) Presence of Burkholderia cepacia complex and others# 20/142 (14.1) Duration of P. aeruginosa colonisation yrs 10.2±5.6 Duration of S. aureus colonisation yrs 11.8±6.1 Presence of Aspergillus fumigatus precipitins 38/116 (32.8) Haematology Neutrophilia >8×109 mm−3 56/138 (40.6) Thrombocytosis >400×109 mm−3 28/138 (20.3) Hypereosinophilia >0.5×109 mm−3 18/138 (13) Lymphopenia <1×109 mm−3 8/138 (5.8) Anaemia¶ 6/138 (4.4) Lymphocytosis >4×109 mm−3 5/138 (3.6) Thrombocytopenia <150×109 mm−3 4/138 (2.9) Biochemistry Elevated CRP 96/137 (70) 5<CRP<30 63/137 (46) 30<CRP 33/137 (24) Hepatic cholestasis 34/140 (24.3) Hypoalbuminaemia <35 g·L−1 28/124 (22.6) Hepatic cytolysis 6/140 (4.3) Hypergammaglobulinaemia >14 g·L−1 75/143 (52.4) Circulating monoclonal Ig+ 29/142 (20.4) Data are presented as n/N available (%) or mean±sd. P. aeruginosa: Pseudomonas aeruginosa; S. aureus: Staphylococcus aureus; CRP: C-reactive protein; Ig: immunoglobulins. #: or heterogeneity restriction; ¶: haemoglobin <11.5 g·dL−1; +: Stenotrophomonas maltophilia.
- Table 3—
Prevalence of antibodies in the cystic fibrosis patients
Subjects n 144 ASCA-IgA >25 IU·mL−1 43.7 ASCA-IgA associated with ASCA-IgG 21.8 ANCA >1/20 40 Anti-BPI ≥1 AU·mL−1 23.6 Anti-PR3, anti-MPO 0 ANA >1/80 25 Anti-dsDNA antibodies ≥5 IU·mL−1 0.7 ENA 0 IgM rheumatoid factor >1.3 AU·mL−1 2.7 Anti-CCP antibodies >25 IU·mL−1 7.6 Anti-CCP associated with IgM rheumatoid factor 1.5 Anti-gliadin IgA >50 IU·mL−1 12.5 Anti-GAD >1 IU·mL−1 2 Anti-IA-2 >0.75 IU·mL−1 1.5 Anti-actin >1/50 1.5 Anti-thyroperoxidase >60 IU·mL−1 2 Data are presented as %, unless otherwise stated. ASCA: anti-Saccharomyces cerevisiae antibodies; Ig: immunoglobulins; ANCA: antineutrophil cytoplasmic antibodies; BPI: bactericidal/permeability-increasing protein; PR3: proteinase-3; MPO: myeloperoxidase; ANA: antinuclear antibodies; ENA: extractable nuclear antigens; CCP: cyclic citrullinated peptide; GAD: glutamic acid decarboxylase; IA-2: Islet antigen-2.
