Chronic granulomatous disease: A multicenter study from the MENA region

To study chronic granulomatous disease (CGD) in the Middle East and North Africa (MENA) region, we conducted a retrospective multicenter study to investigate the clinical features, infections, microorganisms, management, mortality, and survival of patients with CGDs. A total of 322 patients were included; the M:F ratio was 1.8, and the median ages at disease onset and diagnosis were 6.0 and 20.0 mo, respectively. The most common infections included pneumonia (58.0%), lymphadenitis (49.1%), skin abscesses/cellulitis (40.7%), and invasive pulmonary aspergillosis (17.7%). The most commonly isolated microorganisms were Staphylococcus spp. (22%), Aspergillus spp. (19.3%), and Pseudomonas spp. (11.2%). The genetic diagnosis was established in 108 (30.4%) patients: AR inheritance in 84.3% and X-linked in 15.7%. The median survival age is 8.5 years, and the estimated 10-year survival rate is 77.3%. CGD represents a significant disease burden in the MENA region, necessitating early intervention with molecular/genetic testing and HSCT availability.