- is an impaired immune system
- most common symptomatic primary immunodeficiency
- caused by
- mostly unknown
- <10% genetic
- resulting in
- recurrent respiratory tract infections.
- Cellular changes
- serum IgA and IgG: decreased but not absent
- serum IgM: occasionally decreased
- circulating T and B lymphocytes: present
- Organs
- skin
- sinuses
- lung
- bronchiectasis withILD
- (e.g. granulomatous-lymphocytic interstitial lung disease)
- recent term given to describe non-infectious diffuse lung disease complications that have been reported to traditionally develop in common variable immunodeficiency (CVID) patients
- histologic patterns
- bronchiectasis could either be due to repeated lung infection or manifestation of dysregulated lymphoid proliferation 1
- bronchiectasis has been reported as up to 65% of patients according to one study 2
- (e.g. granulomatous-lymphocytic interstitial lung disease)
- ~10% of patients a systemic granulomatous disease
- pulmonary nodules
- ground glass changes
- lung parenchymal opacification: may be multifocal
- other features
- intrathoracic or intra-abdominal lymphadenopathy and/ or hepatosplenomegaly
- bronchiectasis withILD
- liver
- hematologic
-
-
Links and References
- Radiopedia
-
- TCV