Objective: Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immunodeficiency that is characterised by bacteria and fungal infections with defective phagocytosis. Interferongamma (INF-γ) has diverse roles in the innate and adaptive responses. Despite several decades of work on INF-γ treatment in CGD, contraversy remains about its use.
Materials and Methods: Fifty seven patients with CGD from 14 immunology centers were enrolled to our multi-center study. A questionnaire including patients demographic datas and clinical manifestations such as infectious and granulomatous complications up to enrolment was obtained before and after INF-γ therapy.
Results: Fifty seven patients 14 (25%) girls and 34 (75%) boys aged from 2-35 years (mean age: 10.9 ± 7.4 ) were enrolled. The mean age of diagnosis were 4.9 ± 4.8 (0.1-19). 56% of the patient`s family had consanguineous marriage and 60% had a primary immunodeficiency (PID) history. Ninety five of the patients were treated with trimethoprim- sulfamethoxazole (TMP-SMX) and 89.5% of them with itraconazol while 60% of them were received INF-γ treatment. The patients receiving INF-γ therapy tend to have lower infectious complications like severe infections, pneumonia, soft tissue infections and lymphadenitis. Aspergillus infection, tissue abcesses and granulomatous complications were also lower in this group. The annual infectious complications according to CGD subtypes, were also lower in gp91phox with receiving INF-γ therapy.
Conclusion: The demographic and clinical data of CGD patients in our study indicate that INF-γ prophylaxis treatment decreases the infectious and granulomatous complications in some majority of CGD patients especially in gp91phox