Table 2.
Pulmonary complications associated with primary immunodeficiencies.
| PIDs group | Frequency | Pulmonary complication Infectious | Example of PIDs | Other |
|---|---|---|---|---|
| Humoral immunity | 50–60% | Recurrent pneumonia Infections by encapsulated or atypical pathogens |
Airway disease (bronchiectasis or asthma) ILD (LIP, COP, GLILD) |
CVID IgA deficiency XLA |
| Cellular immunity | 5–10% | Recurrent pulmonary infections (Pseudomonas, Pneumocystis jiroveci, CMV, Aspergillus…) | Airway disease (bronchiectasis, BO) Malignancy (lymphoma) |
Wiskott–Aldrich DiGeorge |
| Combined ID (humoral + cellular) | 20% | Opportunistic infections in childhood (CMV, P. jiroveci, Microbacteria…) | ILD Malignancy (lymphoma, leukemia) |
SCID Ataxia telangiectasia |
| Phagocyte disorders | 10–15% | Recurrent pulmonary infections (S. aureus, Klebsiella, Serratia, Nocardia, Aspergillus…) Necrotizing pneumonia Pulmonary abscess Empyema |
Autoimmune disease Antiphospholipid syndrome ILD (granulomatosis) |
CGD Chédiak-Higashi |
| Complement deficiency | 2% | Infections by encapsulated pathogens (S. Pneumoniae, H. Influenzae, N. Meningitidis) | Autoimmune disease (vasculitis, SLE) | Complement deficiency |
CGD, chronic granulomatous disease; CMV, cytomegalovirus; COP, Cryptogenic organizing pneumonia; CVID, common variable immunodeficiency; GLILD, granulomatous-lymphocytic interstitial lung disease; ID, immunodeficiency; ILD, diffuse interstitial lung disease; LIP, lymphoid interstitial pneumonia; OB, obliterative bronchiolitis; PIDs, primary immunodeficiency; SCID, severe combined immunodeficiency; SLE, Systemic lupus erythematosus; XLA, X-linked agammaglobulinemia.