Imaging findings of pulmonary manifestations of chronic granulomatous disease in a large single center from Shanghai, China (1999–2018)

Chronic granulomatous disease (CGD) is characterized by recurrent infections and granuloma formation in multiple organs, especially the lung. We aimed to investigate pulmonary manifestations by computed tomography (CT). In total, 100 patients with 117 episodes of pulmonary infection were included. Chest CT scans of every episode were analyzed. Random nodules were the most common findings (79.49%), followed by ground-grass opacities (74.36%), focal consolidations (62.39%), and masses (59.83%). Cavities (12.82%) and multiple small abscesses (17.09%) could be found in the consolidations and masses. CT revealed interstitial pneumonia with tree-in-bud opacities (17.09%), interlobular septal thickening (23.08%) and emphysema (35.04%), which were more severe in the bilateral upper lobes. Mediastinal and hilar lymphadenopathy (78.63%) and axillary lymphadenopathy (65.81%) were common. Fungal infection (n = 27) was the most common and presented with multiple nodules and masses. Approximately 1/4 of fungal infections had interstitial pneumonia. In Staphylococcus aureus (n = 6) and Klebsiella pneumoniae (n = 3) infections, large areas of consolidation were common. In tuberculosis infection, the pulmonary infections were more severe and complex. For Bacillus Calmette-Guérin disease, left-sided axillary lymphadenopathy was a characteristic manifestation. CT images of CGD demonstrated variable pulmonary abnormalities. The main infectious organisms have unique imaging features.