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