PT  - JOURNAL ARTICLE
AU  - Hélène Salvator
AU  - Nizar Mahlaoui
AU  - Emilie Catherinot
AU  - Elisabeth Rivaud
AU  - Benoit Pilmis
AU  - Raphael Borie
AU  - Bruno Crestani
AU  - Colas Tcherakian
AU  - Felipe Suarez
AU  - Bertrand Dunogue
AU  - Marie-Anne Gougerot-Pocidalo
AU  - Margarita Hurtado-Nedelec
AU  - Jean-François Dreyfus
AU  - Isabelle Durieu
AU  - Fanny Fouyssac
AU  - Olivier Hermine
AU  - Olivier Lortholary
AU  - Alain Fischer
AU  - Louis-Jean Couderc
TI  - Pulmonary manifestations in adult patients with chronic granulomatous disease
AID  - 10.1183/09031936.00118414
DP  - 2015 Jun 01
TA  - European Respiratory Journal
PG  - 1613--1623
VI  - 45
IP  - 6
4099  - http://erj.ersjournals.com/content/45/6/1613.short
4100  - http://erj.ersjournals.com/content/45/6/1613.full
SO  - Eur Respir J2015 Jun 01; 45
AB  - Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. The disease is characterised by recurrent infections and inflammatory events, frequently affecting the lungs. Improvement of life expectancy now allows most patients to reach adulthood. We aimed to describe the pattern of pulmonary manifestations occurring during adulthood in CGD patients. This was a retrospective study of the French national cohort of adult patients (≥16 years old) with CGD. Medical data were obtained for 67 adult patients. Pulmonary manifestations affected two-thirds of adult patients. Their incidence was significantly higher than in childhood (mean annual rate 0.22 versus 0.07, p=0.01). Infectious risk persisted despite anti-infectious prophylaxis. Invasive fungal infections were frequent (0.11 per year per patient) and asymptomatic in 37% of the cases. They often required lung biopsy for diagnosis (10 out of 30). Noninfectious respiratory events concerned 28% of adult patients, frequently associated with a concomitant fungal infection (40%). They were more frequent in patients with the X-linked form of CGD. Immune-modulator therapies were required in most cases (70%). Respiratory manifestations are major complications of CGD in adulthood. Noninfectious pulmonary manifestations are as deleterious as infectious pneumonia. A specific respiratory monitoring is necessary. Pulmonary involvement is a major concern in adult CGD patients, making specific respiratory monitoring necessary http://ow.ly/FjaRS