Table 4.

Warning signs of primary immunodeficiency for pulmonologists.

Clinical events	PID	Laboratory tests (*see Table 2)
Pneumonia due to extracellular bacteria + otitis and sinusitis	Antibody deficiencies Complement deficiencies	Screening for antibody-mediated immunity (AMI) C, ANA
Pulmonary abscess Pneumatocele	Chronic granulomatous disease (CGD) Hyper IgE syndrome (HIES) Characteristics: S. aureus pneumonia, eczema, fungal infection, joint hypermobility, rough facial features	Serum IgE, eosinophilia Specific score
Pneumonia due to Staphylococcus or fungi	Chronic granulomatous disease (CGD): susceptibility to infections by catalase positive microorganisms. Other infections: adenitis, liver abscess, osteomyelitis	P
	Glucose-6-phosphate dehydrogenase (G6PD) deficiency Myeloperoxidase deficiency (common in diabetes)	G6PD activity Peroxidase level
	HIES	Serum IgE, eosinophilia Specific score
Pneumonia caused by P. jirovecii	T-cell deficiencies/CD4 lymphopenia	Screening for cell-mediated immunity (CMI), AMI Lymphoproliferation test
	CD40 ligand (L) deficiency	CMI, AMI
	Wiskott-Aldrich syndrome (WAS), eczema + thrombocytopenia	CBC including number and size of platelets, CMI, AMI
Pneumonia caused by Mycobacterium tuberculosis or atypical mycobacteria	T-cell deficiencies/CD40L deficiency Mendelian susceptibility to mycobacterial diseases	CMI, AMI II

Adapted from Costa-Carvalho et al.⁵