Thoracic Manifestations of Primary Immunodeficiency Disorders.

ABSTRACT

Primary immunodeficiency disorders (PIDD) are a group of disorders presenting with recurrent infections. The authors retrospectively reviewed the imaging records of 24 proven cases of PIDD and correlated the imaging findings with the type of defect. Final diagnoses were categorized in four groups; Group I (humoral immunodeficiency), Group 2 (cell mediated immunodeficiency), Group 3 (phagocytic disorders) and Group 4 (others). Group 3 was the commonest, followed by 1 and 2. Three most common disorders encountered were chronic granulomatous disease (CGD) (7/24), hyper IgE syndrome (5/24) and common variable immunodeficiency (CVID) (4/24). Consolidation (12/24), nodules (10/24), bronchiectasis (9/24) and lymphadenopathy (8/24) were the commonest imaging findings. Although not statistically significant, some definite imaging trends could be established. Presence of consolidation and nodules; with absence of bronchiectasis was the striking finding in Group 3. Group 2 disorders predominantly showed bronchiectasis, whereas presence of consolidation, nodules, bronchiectasis all were very common in Group 1.