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Comparison of different sets of immunological tests to identify treatable immunodeficiencies in adult bronchiectasis patients

Stefano Aliberti, Francesco Amati, Andrea Gramegna, Barbara Vigone, Martina Oriano, Giovanni Sotgiu, Marco Mantero, Edoardo Simonetta, Laura Saderi, Anna Stainer, Serena Tammaro, Paola Marchisio, Eva Polverino, James D. Chalmers, Francesco Blasi
ERJ Open Research 2022 8: 00388-2021; DOI: 10.1183/23120541.00388-2021
Stefano Aliberti
1Dept of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy
2IRCCS Humanitas Research Hospital, Respiratory Unit, Rozzano, Italy
10These authors contributed equally
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  • For correspondence: stefano.aliberti@hunimed.eu
Francesco Amati
1Dept of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy
2IRCCS Humanitas Research Hospital, Respiratory Unit, Rozzano, Italy
10These authors contributed equally
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Andrea Gramegna
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
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Barbara Vigone
5Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy
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Martina Oriano
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
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Giovanni Sotgiu
6Clinical Epidemiology and Medical Statistics Unit, Dept of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy
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Marco Mantero
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
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Edoardo Simonetta
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
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Laura Saderi
6Clinical Epidemiology and Medical Statistics Unit, Dept of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy
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Anna Stainer
1Dept of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy
2IRCCS Humanitas Research Hospital, Respiratory Unit, Rozzano, Italy
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Serena Tammaro
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
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Paola Marchisio
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
7Pediatric Highly Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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Eva Polverino
8Pneumology Dept, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Barcelona, Spain
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James D. Chalmers
9College of Medicine, University of Dundee, Dundee, UK
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Francesco Blasi
3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Respiratory Unit and Cystic Fibrosis Adult Center, Milan, Italy
4Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
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Tables

  • TABLE 1

    Demographics, disease severity, clinical, radiological, functional and microbiological characteristics of the study population

    VariablesStudy population (n=401)
    Demographics
     Female sex317 (79.1%)
     Age, years63 (50–71)
     Body mass index, kg·m−221.5 (19.5–24.0)
     Underweight58 (14.6%)
     Former or current smoker180 (44.9%)
    Comorbidities
     GORD180 (44.9%)
     Rhinosinusitis138 (34.4%)
     Cardiovascular diseases142 (35.4%)
     Systemic hypertension93 (23.2%)
     Asthma60 (15.0%)
     Osteoporosis69 (17.2%)
     COPD32 (8.0%)
     Depression34 (8.5%)
     Anxiety27 (6.7%)
     History of neoplastic disease56 (14.0%)
     Diabetes17 (4.2%)
     BACI score0 (0–3)
    Functional evaluation
     FEV1, % pred87 (71–101)
     FEV1 <50% pred30 (7.9)
     FVC, % pred, mean±sd97.5±21.7
    Microbiology
     Chronic infection120 (35.9%)
     Chronic Pseudomonas aeruginosa infection76 (22.7%)
     Chronic Haemophilus influenzae infection20 (6.0%)
     NTM-PD41 (12.2%)
    Clinical status
     Exacerbations2 (1–3)
     ≥3 exacerbations in the previous year132 (32.9%)
     LTOT19 (4.7%)
     Daily sputum267 (66.6%)
     Sputum volume, mL6 (4–20)
    Chronic treatment
     Chronic macrolide therapy40 (10.0%)
     Chronic antibiotic inhaled therapy21 (5.2%)
    Radiology
     Reiff score4 (2–6)
     Number of lobes involved3 (2–5)
    Disease severity
     BSI score6 (3–9)
     BSI moderate–severe244 (63.9%)
     BSI severe98 (25.7%)
     FACED score2 (1–3)
     FACED moderate–severe141 (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)

      S1S2S3S4S5
      Any immunodeficiency36 (8.9%)93 (23.2%)179 (44.6%)179 (44.6%)179 (44.6%)
      Primary immunodeficiency29 (7.2%)83 (20.7%)158 (39.4%)158 (39.4%)158 (39.4%)
       Isolated IgG subclass deficiency0036 (9%)36 (9%)36 (9%)
      •   Isolated IgG1 subclasses deficiency

      003 (0.8%)3 (0.8%)3 (0.8%)
      •   Isolated IgG2 subclasses deficiency

      002 (0.5%)2 (0.5%)2 (0.5%)
      •   Isolated IgG3 subclasses deficiency

      006 (1.5%)6 (1.5%)6 (1.5%)
      •   Isolated IgG4 subclasses deficiency

      0024 (5.9%)24 (5.9%)24 (5.9%)
      •   IgG1 and IgG3 subclasses deficiency

      001 (0.2%)1 (0.2%)1 (0.2%)
       Isolated IgM deficiency003 (0.7%)3 (0.7%)3 (0.7%)
       Unclassified antibody deficiency006 (1.5%)6 (1.5%)6 (1.5%)
       CVID002 (0.5%)2 (0.5%)2 (0.5%)
       Severe combined immunodeficiency00000
       Combined immunodeficiency00000
       Hyper-IgE syndrome00000
       Isolated IgA deficiency2 (0.5%)2 (0.5%)2 (0.5%)2 (0.5%)2 (0.5%)
       DiGeorge Syndrome1 (0.2%)1 (0.2%)1 (0.2%)1 (0.2%)1 (0.2%)
       Unclassified immunodeficiency26 (6.5%)80 (20%)108 (26.9%)108 (26.9%)108 (26.9%)
      Secondary immunodeficiency7 (1.7%)10 (2.5%)21 (5.2%)21 (5.2%)21 (5.2%)
       Immunosuppressive drugs1 (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 malignancy6 (1.5%)6 (1.5%)7 (1.7%)7 (1.7%)7 (1.7%)
       Transplant01 (0.2%)3 (0.7%)3 (0.7%)3 (0.7%)
      Other aetiologies113 (28.2%)113 (28.2%)67 (16.7%)67 (16.7%)67 (16.7%)#
      Idiopathic bronchiectasis252 (62.8%)195 (48.6%)155 (38.7%)155 (38.7%)155 (38.7%)
      Treatable immunodeficiencies15 (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)

        VariablesGroup A (n=158)Group B (n=21)Group C (n=155)p-value
        Demographics
         Female sex122 (77.2%)13 (61.9%)129 (83.2%)0.06
         Age, years65 (52–73)70 (65–73)62 (48–70)0.009#
         Body mass index, kg·m−221.1 (19.2–24.0)23.5 (21.9–25.5)21.4 (19.7–24.0)0.08
         Underweight27 (17.2%)2 (10.0%)20 (13.0%)0.48
         Former or current smoker63 (39.9%)13 (61.9%)77 (49.7%)0.07
        Comorbidities
         GORD71 (44.9%)11 (52.4%)74 (47.7%)0.76
         Rhinosinusitis58 (36.7%)7 (33.3%)45 (29.0%)0.35
         Cardiovascular diseases66 (41.8%)12 (57.1%)43 (27.7%)0.004¶
         Systemic hypertension43 (27.2%)8 (38.1%)29 (18.7%)0.06
         Asthma24 (15.2%)1 (4.8%)29 (18.7%)0.25
         Osteoporosis24 (15.2%)10 (47.6%)26 (16.8%)0.001+
         COPD16 (10.1%)2 (9.5%)9 (5.8%)0.32
         Depression17 (10.8%)3 (14.3%)10 (6.5%)0.24
         Anxiety11 (7.0%)2 (9.5%)11 (7.1%)0.84
         History of neoplastic disease23 (14.6%)12 (57.1%)13 (8.4%)<0.0001§
         Diabetes6 (3.8%)7 (33.3%)3 (1.9%)<0.0001ƒ
        Functional
         FEV1, % pred85.9±24.984.6±27.285.6±20.80.97
         FEV1 <50% pred13 (8.8%)2 (10.0%)9 (6.2%)0.62
         FVC, % pred98.5±23.297.5±25.197.3±18.60.89
        Microbiological
         Chronic infection49 (36.6%)5 (29.4%)46 (35.4%)0.84
         Chronic Pseudomonas aeruginosa  infection31 (23.1%)3 (17.7%)30 (23.1%)0.95
         Chronic Haemophilus influenzae infection7 (5.3%)3 (17.7%)8 (6.2%)0.14
         NTM-PD18 (13.4%)3 (17.7%)11 (8.5%)0.29
        Clinical
         Exacerbations2 (1–3)1 (0–3)2 (1–3)0.97
         ≥3 exacerbations in the previous year51 (32.3%)8 (38.1%)49 (31.6%)0.84
         LTOT11 (7.0%)2 (9.5%)4 (2.6%)0.09
         Daily sputum104 (65.8%)15 (71.4%)107 (69.0%)0.77
         Sputum volume, mL7 (4.5–20.0)5.5 (3–50)6 (5–20)0.93
         Chronic macrolide therapy16 (10.1)1 (4.8)11 (7.1)0.62
         Chronic inhaled antibiotic therapy8 (5.1)1 (4.8)10 (6.5)0.86
        Radiological
         Reiff score4 (2–6)4 (2–6)4 (3–6)0.99
         Number of lobes involved3 (2–4)4 (2–6)4 (2–5)0.59
        Disease severity
         BSI score6 (4–9)6 (3.0–8.5)6 (3–8)0.11
         FACED score2 (1–3)2 (2–3)2 (1–3)0.06
         BACI score0 (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.

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        Comparison of different sets of immunological tests to identify treatable immunodeficiencies in adult bronchiectasis patients
        Stefano Aliberti, Francesco Amati, Andrea Gramegna, Barbara Vigone, Martina Oriano, Giovanni Sotgiu, Marco Mantero, Edoardo Simonetta, Laura Saderi, Anna Stainer, Serena Tammaro, Paola Marchisio, Eva Polverino, James D. Chalmers, Francesco Blasi
        ERJ Open Research Jan 2022, 8 (1) 00388-2021; DOI: 10.1183/23120541.00388-2021

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        Comparison of different sets of immunological tests to identify treatable immunodeficiencies in adult bronchiectasis patients
        Stefano Aliberti, Francesco Amati, Andrea Gramegna, Barbara Vigone, Martina Oriano, Giovanni Sotgiu, Marco Mantero, Edoardo Simonetta, Laura Saderi, Anna Stainer, Serena Tammaro, Paola Marchisio, Eva Polverino, James D. Chalmers, Francesco Blasi
        ERJ Open Research Jan 2022, 8 (1) 00388-2021; DOI: 10.1183/23120541.00388-2021
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