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. 2018 Nov 12;19:219. doi: 10.1186/s12931-018-0923-8

Table 2.

Common respiratory manifestations by PID type

Common respiratory manifestations by PID type N (Composition of panel) Votes in agreement (%) Degree of agreement
Predominantly T cell deficiency (combined and PID-related syndromes)
 Recurrent bronchitis 43 (AI, AP, PI, PP) 72.1 Majority
 Idiopathic bronchiectasis 43 (AI, AP, PI, PP) 76.7 Majority
 Recurrent pneumonias* 43 (AI, AP, PI, PP) 88.4 Consensus
 Repeated pneumonia in child 43 (AI, AP, PI, PP) 88.4 Consensus
 Chronic bronchial infection 43 (AI, AP, PI, PP) 79.1 Majority
 Prolonged antibiotic treatment with poor response 43 (AI, AP, PI, PP) 86.0 Consensus
 Abscess and pneumatocele 43 (AI, AP, PI, PP) 55.8 Discrepancy
 Infections caused by rare microorganisms 43 (AI, AP, PI, PP) 100.0 Unanimity
 Pneumonitis or bronchitis with hospitalization in infants 43 (AI, AP, PI, PP) 86.0 Consensus
 Interstitial lung disease 43 (AI, AP, PI, PP) 93.0 Consensus
 Bronchiolitis obliterans 43 (AI, AP, PI, PP) 67.4 Majority
 Alveolar proteinosis 43 (AI, AP, PI, PP) 76.7 Majority
 Absent thymus or aplasia 43 (AI, AP, PI, PP) 95.3 Consensus
Antibody deficiencies
 Recurrent bronchitis 43 (AI, AP, PI, PP) 90.7 Consensus
 Idiopathic bronchiectasis 43 (AI, AP, PI, PP) 97.7 Consensus
 Recurrent pneumonias* 43 (AI, AP, PI, PP) 97.7 Consensus
 Repeated pneumonia in child 43 (AI, AP, PI, PP) 97.7 Consensus
 Chronic bronchial infection 43 (AI, AP, PI, PP) 90.7 Consensus
 Prolonged antibiotic treatment with poor response 43 (AI, AP, PI, PP) 97.7 Consensus
 Pneumonia due to encapsulated bacteria 43 (AI, AP, PI, PP) 97.7 Consensus
 Interstitial lung disease 43 (AI, AP, PI, PP) 79.1 Majority
 Bronchiolitis obliterans 43 (AI, AP, PI, PP) 60.5 Discrepancy
 Pulmonary lymphoma 43 (AI, AP, PI, PP) 69.8 Majority
 Thymoma 43 (AI, AP, PI, PP) 88.4 Consensus
Immune deregulation
 Recurrent bronchitis 43 (AI, AP, PI, PP) 69.8 Majority
 Idiopathic bronchiectasis 43 (AI, AP, PI, PP) 74.4 Majority
 Chronic bronchial infection 43 (AI, AP, PI, PP) 65.1 Majority
 Pneumonia due to encapsulated bacteria 43 (AI, AP, PI, PP) 67.4 Majority
 Interstitial lung disease 43 (AI, AP, PI, PP) 81.4 Consensus
Phagocyte disorders
 Recurrent pneumonias* 43 (AI, AP, PI, PP) 90.7 Consensus
 Prolonged antibiotic treatment with poor response 43 (AI, AP, PI, PP) 88.4 Consensus
 Abscess and pneumatocele 43 (AI, AP, PI, PP) 97.7 Consensus
 Infections caused by rare microorganisms 43 (AI, AP, PI, PP) 97.7 Consensus
 Alveolar proteinosis 43 (AI, AP, PI, PP) 69.8 Majority
Innate immunity disorder
 Recurrent pneumonias* 43 (AI, AP, PI, PP) 97.7 Consensus
 Pneumonia due to encapsulated bacteria 43 (AI, AP, PI, PP) 95.3 Consensus
 Abscess and pneumatocele 43 (AI, AP, PI, PP) 83.7 Consensus
 Infections caused by rare microorganisms 43 (AI, AP, PI, PP) 90.7 Consensus
Complement deficiency
 Recurrent bronchitis 43 (AI, AP, PI, PP) 65.1 Majority
 Idiopathic bronchiectasis 43 (AI, AP, PI, PP) 72.1 Majority
 Recurrent pneumonias* 43 (AI, AP, PI, PP) 83.7 Consensus
 Repeated pneumonia in child 43 (AI, AP, PI, PP) 83.7 Consensus
 Chronic bronchial infection 43 (AI, AP, PI, PP) 67.4 Majority
Autoinflammatory disease
 Recurrent serositis 43 (AI, AP, PI, PP) 100.0 Unanimity

AI: adult immunologists; AP: adult pulmonologists; PI: pediatric immunologists; PID: primary immunodeficiency disease; PP: pediatric pulmonologists

* One pneumonia per year for more than one year