Table 5 Respiratory tests to be conducted in adult (consensus) and pediatric (no consensus) patients with suspected PID
From: Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management
First levela | Second levelb | Third levelc | N (Composition of panel) | Votes in agreement (%) | Degree of agreement |
|---|---|---|---|---|---|
Adults | |||||
Refer to pulmonologist when warning signs detected | Spirometry* Plethysmography DLCO HRCT +/− abdominal ultrasound Sputum culture (bacteria, mycobacteria, and fungi), if expectoration | Exercise stress tests. Bronchoscopy with bronchoalveolar lavage and transbronchial cryobiopsy. Open lung biopsy or by video-assisted thoracoscopy in case of interstitial lung disease. | 5 (AP) | 100.0 | Unanimity |
Children | |||||
Spirometry, if possible according to patient age and availability Chest X-ray | Spirometry (technically possible ≥3 years) Plethysmography (≥ 6–7 years, if available) DLCO (≥ 6–7 years) Lung HRCT +/− abdominal ultrasound Sputum culture, if expectoration (induced sputum, if no expectoration) | Lung function studies by age: • In infants: plethysmography; tidal volume determination; tidal or raised volume rapid thoracoabdominal compression technique. • In preschoolers: oscillometry; oscillatory resistance. Bronchoscopy with bronchoalveolar lavage, in the case of localized or diffuse bronchiectasis or interstitial lung disease, even if patient is expectorating. Open lung biopsy or video-assisted thoracoscopy in case of interstitial lung disease Transbronchial cryobiopsy in children > 7 years as alternative to lung biopsy | 4 (PP) | 50.0 | Discrepancy |