RT Journal Article
SR Electronic
T1 Pulmonary manifestations in adult patients with chronic granulomatous disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1613
OP 1623
DO 10.1183/09031936.00118414
VO 45
IS 6
A1 Hélène Salvator
A1 Nizar Mahlaoui
A1 Emilie Catherinot
A1 Elisabeth Rivaud
A1 Benoit Pilmis
A1 Raphael Borie
A1 Bruno Crestani
A1 Colas Tcherakian
A1 Felipe Suarez
A1 Bertrand Dunogue
A1 Marie-Anne Gougerot-Pocidalo
A1 Margarita Hurtado-Nedelec
A1 Jean-François Dreyfus
A1 Isabelle Durieu
A1 Fanny Fouyssac
A1 Olivier Hermine
A1 Olivier Lortholary
A1 Alain Fischer
A1 Louis-Jean Couderc
YR 2015
UL http://erj.ersjournals.com/content/45/6/1613.abstract
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