Pulmonary manifestations of chronic granulomatous disease
Patient presenting for follow up of pneumonia with known PMH of chronic granulomatous disease with recurrent aspergillus and Staph Aureus pneumonia
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Severe thick-walled cystic changes in the bilateral upper lobes with moderate bronchiectasis and associated fibrosis and pleural thickening. Multiple areas of nodular consolidation scattered throughout the lungs, the largest within the right lower lobe and measuring up to 2 cm in size. Moderate tree-in-bud nodularity throughout the mid and upper lungs. Mild mosaic attenuation. Multiple sub-centimeter scattered granulomas seen through bilateral lungs.
Chronic granulomatous disease is an immunodeficiency due to inability to produce free oxygen radicals because of an inactive NADPH oxidase enzyme. These patients have increased susceptibility to infections with catalase positive organisms (S. Aureus, Candida, Aspergillus). Along with the sequelae of repeated opportunistic infections, granulomatous deposits are seen throughout the body including the lung, spleen and liver.
This case identifies a relatively young male whose lungs have been subject to repeated infections and the sequelae of CGD (bronchiectasis, scarring, cystic changes and scattered granulomas). The patient was recovering from a pneumonia which was lower lobe predominant. The increased scarring and manifestations of the disease being concentrated in the upper lobes and the apices may be related to his repeated bouts of Aspergillus infection which is upper lobe predominant.
- Hélène Salvator, Nizar Mahlaoui, Emilie Catherinot, Elisabeth Rivaud, Benoit Pilmis, Raphael Borie, Bruno Crestani, Colas Tcherakian, Felipe Suarez, Bertrand Dunogue, Marie-Anne Gougerot-Pocidalo, Margarita Hurtado-Nedelec, Jean-François Dreyfus, Isabelle Durieu, Fanny Fouyssac, Olivier Hermine, Olivier Lortholary, Alain Fischer, Louis-Jean Couderc. Pulmonary manifestations in adult patients with chronic granulomatous disease. (2015) European Respiratory Journal. 45 (6): 1613. doi:10.1183/09031936.00118414 - Pubmed