Fiche publication
Date publication
juin 2015
Journal
The European respiratory journal
Auteurs
Membres identifiés du Cancéropôle Est :
Dr FOUYSSAC Fanny
Tous les auteurs :
Salvator H, Mahlaoui N, Catherinot E, Rivaud E, Pilmis B, Borie R, Crestani B, Tcherakian C, Suarez F, Dunogue B, Gougerot-Pocidalo MA, Hurtado-Nedelec M, Dreyfus JF, Durieu I, Fouyssac F, Hermine O, Lortholary O, Fischer A, Couderc LJ
Lien Pubmed
Résumé
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.
Mots clés
Adolescent, Adult, Anti-Infective Agents, therapeutic use, Asymptomatic Diseases, Biopsy, Cohort Studies, Female, Granulomatous Disease, Chronic, complications, Humans, Immunologic Factors, therapeutic use, Lung, pathology, Lung Diseases, etiology, Lung Diseases, Fungal, diagnosis, Male, Membrane Glycoproteins, genetics, Middle Aged, NADPH Oxidase, genetics, Pneumonia, Bacterial, diagnosis, Retrospective Studies, Young Adult
Référence
Eur. Respir. J.. 2015 Jun;45(6):1613-23
