Granulomatous-lymphocytic interstitial lung disease associated with common variable immunodeficiency - CT findings

Purpose: To evaluate computed tomography (CT) scans of individuals with granulomatous-lymphocytic interstitial lung disease and common variable immunodeficiency (CVID) to determine if there are imaging features that distinguish this manifestation of CVID from the more usual imaging findings. Materials and Methods: A review of the CVID population at our institution identified a series of 5 patients with CVID who had documented granulomatous disease on biopsy specimens. The initial and follow-up CT examinations were reviewed by 2 radiologists, and imaging findings in the chest and abdomen were tabulated by consensus. In addition, a pathologist reviewed histopathologic specimens and clinical presentations and therapeutic interventions were obtained from patient charts. Results: In all, 515 patients (100%) had widespread pulmonary micronodules with a lower lung zone predominance, 415 (80%) had smooth interlobular septal thickening with mid to lower lung zone predominance, 115 (20%) had mild bronchiectasis, 4/5 (80%) had multifocal pulmonary consolidation, 515 (100%) had thoracic or abdominal lymphadenopathy, 2/15 (40%) had hepatomegaly, 5/5 (100%) had splenomegaly, 1/5 (20%) had nonspecific hypoattenuating splenic lesions, and 2/5 (40%) had nonspecific hypoattenuating renal lesions. The pulmonary nodules and lymphadenopathy commonly tended to wax and wane in severity over time, and more marked disease was often associated with areas of focal consolidation. Conclusion: Grarmlomatous-lymphocytic interstitial lung disease, which can occur in patients with CVID, presents with CT findings distinct from the usual airway abnormalities most commonly associated with CVID.