Background/Objectives: CGD is a rare primary immunodeficiency disorder with a propensity for opportunistic filamentous fungi, but Aspergillus infection is the most common. Infection with Aspergillus species can cause either allergic or invasive pulmonary disease in susceptible individuals. We aimed to study allergic or invasive pulmonary aspergillosis among CGD patients at the main referral center for immunological disorders in Iran.
Methods: Eighteen CGD patients suspected to pulmonary aspergillosis on the basis of the lung HRCT (high resolution CT scan) were entered to this study from 2005 to 2008. Allergic bronchopulmonary aspergillosis (ABPA) was considered with the following criteria: 1-asthma, 2-current or previous compatible pulmonary infiltrates, 3-elevated total serum IgE 4-elevated specific serum IgE and IgG antibodies to Aspergillus, and 5-centeral bronchiectasis. In addition, Aspergillus culture and smear of BAL/sputum were done for the patients and EROTC/MSG criteria were considered for diagnosis of invasive pulmonary aspergillosis.
Results: Sixteen cases (88.9%) had pulmonary aspergillosis; 5 patients (27.8%) had been sensitized to Aspergillus (positive ABPA) and 11 patients (61.1%) had invasive pulmonary aspergillosis. Aspergillus culture was positive in 14 cases; A. fumigatus in 12 cases and A. flavous in 2 cases.
Conclusions: Aspergillus infection is one of the most important causes of morbidity and mortality in CGD patients and pulmonary aspergillosis should be considered in all these patients with pulmonary problems. Although ABPA is not typically associated with invasive disease but early diagnosis and treatment is crucial for preventing to become invasive.