Single or multiple pneumatoceles: May expand when superinfected with bacteria and fungi
Wiskott-Aldrich syndrome (WAS)
X-linked recessive immunodeficiency disorder: Eczema, thrombocytopenia with small defective platelets, recurrent infections
Prolonged bleeding from circumcision site, bruising, or bloody diarrhea during infancy
Pyogenic infections before 1st year of age: Meningitis, otitis media, pneumonia, sepsis
Most common pathogens: S. pneumoniae, P. jirovecii, and herpes viruses
Massive bleeding, infection, vasculitis, autoimmune cytopenias, or lymphoreticular malignancies are common causes of death
Phagocytic defects
Chronic granulomatous disease (CGD)
Defect in membrane-associated nicotinamide adenine dinucleotide phosphate oxidase in phagocytic cells
Onset in infancy, childhood, or, less commonly, early adolescence
Male:female ratio of 6:1
Recurrent bronchopneumonia, empyema, and lung abscess
Most common pathogens: S. aureus, Klebsiella, Pseudomonas, Aspergillus, and Candida
Chédiak-Higashi syndrome (CHS)
Rare autosomal recessive defect
Impaired microbicidal activity of phagocytes
Up to 30% of affected patients have segmental or lobar pneumonia
S. aureus, H. influenzae, group A streptococcus, and gram-negative organisms (Klebsiella, Pseudomonas)
Acute respiratory failure
Extensive histiocytic infiltration of lungs
Other immunodeficiencies
Chronic mucocutaneous candidiasis (CMC)
Phenotypic manifestation of complex immunodeficiency
Recurrent or persistent Candida infections of skin, nails, oropharynx, and lung
Risk of developing bronchiectasis in early childhood
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
CLINICAL ISSUES
Presentation
Demographics
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Freeman AF et al: Hyper-IgE syndromes and the lung. Clin Chest Med. 37(3):557-67, 2016
Wu EY et al: Clinical and imaging considerations in primary immunodeficiency disorders: an update. Pediatr Radiol. 46(12):1630-1644, 2016
Nonas S: Pulmonary Manifestations of primary immunodeficiency disorders. Immunol Allergy Clin North Am. 35(4):753-66, 2015
Touw CM et al: Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allergy Immunol. 21(5):793-805, 2010
Tanaka N et al: Lung diseases in patients with common variable immunodeficiency: chest radiographic, and computed tomographic findings. J Comput Assist Tomogr. 30(5):828-38, 2006
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Primary immunodeficiency disorders (PIDD)
Definitions
Heterogeneous group of genetic diseases involving immune system
> 300 distinct disorders described
Disorders of antibody function
Selective IgA deficiency (SIgAD)
Most common of all inborn defects of humoral immunity
Isolated absence or near absence of serum and secretory IgA
Serum IgA < 5 mg/dL
> 1% of recurrent infections in children
Pneumonia: Streptococcus pneumoniae, Haemophilus influenzae, Mycoplasma species, enterovirus species
Single or multiple pneumatoceles: May expand when superinfected with bacteria and fungi
Wiskott-Aldrich syndrome (WAS)
X-linked recessive immunodeficiency disorder: Eczema, thrombocytopenia with small defective platelets, recurrent infections
Prolonged bleeding from circumcision site, bruising, or bloody diarrhea during infancy
Pyogenic infections before 1st year of age: Meningitis, otitis media, pneumonia, sepsis
Most common pathogens: S. pneumoniae, P. jirovecii, and herpes viruses
Massive bleeding, infection, vasculitis, autoimmune cytopenias, or lymphoreticular malignancies are common causes of death
Phagocytic defects
Chronic granulomatous disease (CGD)
Defect in membrane-associated nicotinamide adenine dinucleotide phosphate oxidase in phagocytic cells
Onset in infancy, childhood, or, less commonly, early adolescence
Male:female ratio of 6:1
Recurrent bronchopneumonia, empyema, and lung abscess
Most common pathogens: S. aureus, Klebsiella, Pseudomonas, Aspergillus, and Candida
Chédiak-Higashi syndrome (CHS)
Rare autosomal recessive defect
Impaired microbicidal activity of phagocytes
Up to 30% of affected patients have segmental or lobar pneumonia
S. aureus, H. influenzae, group A streptococcus, and gram-negative organisms (Klebsiella, Pseudomonas)
Acute respiratory failure
Extensive histiocytic infiltration of lungs
Other immunodeficiencies
Chronic mucocutaneous candidiasis (CMC)
Phenotypic manifestation of complex immunodeficiency
Recurrent or persistent Candida infections of skin, nails, oropharynx, and lung
Risk of developing bronchiectasis in early childhood
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
CLINICAL ISSUES
Presentation
Demographics
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Freeman AF et al: Hyper-IgE syndromes and the lung. Clin Chest Med. 37(3):557-67, 2016
Wu EY et al: Clinical and imaging considerations in primary immunodeficiency disorders: an update. Pediatr Radiol. 46(12):1630-1644, 2016
Nonas S: Pulmonary Manifestations of primary immunodeficiency disorders. Immunol Allergy Clin North Am. 35(4):753-66, 2015
Touw CM et al: Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allergy Immunol. 21(5):793-805, 2010
Tanaka N et al: Lung diseases in patients with common variable immunodeficiency: chest radiographic, and computed tomographic findings. J Comput Assist Tomogr. 30(5):828-38, 2006
STATdx includes over 200,000 searchable images, including x-ray, CT, MR, and ultrasound images. To access all images, please log in or subscribe.