The Efficacy of Long-Term Voriconazole Prophylaxis on Seven Patients With Chronic Granulomatous Disease and Review of the Literature

Abstract

Objective: Chronic granulomatous disease (CGD) a primary immunodeficiency disease that affects the innate immune system, and characterized by life-threatening and/or recurrent infections caused by bacteria and fungi. Invasive fungal diseases (IFDs) are the most common causes of death among CGD patients. Itraconazole is usually used for prophylaxis in CGD patients at high risk of IFD. However, patient compliance with oral itraconazole use is low due to gastrointestinal side effects. We aim to report the efficacy of long-term voriconazole prophylaxis for IFD on our CGD patients. Material and Methods: This retrospective study includes seven CGD patients. All data were recorded from their hospital registries. CGD was diagnosed with both functional and genetic analysis. Patients who had used voriconazole prophylaxis for six months or more after clinical and/or laboratory diagnosis of IFD were included in this study. Invasive fungal diseases were classified as proven, probable, or possible according to the revised criteria of the guideline. Results:All seven patients were male. Two had an X-linked recessive subtype of CGD (X-CGD), and the others had autosomal recessive subtypes of CGD. The median age at diagnosis was 7 (4.5-19) years. Aspergillus spp was showed in three of patients. The median age at which first IFD oc curred was 10 (4.5-19) years. The median follow-up period was 6 (3-16) years. After voriconazole prophylaxis, 6 patients did not suffer from IFD second time. Only one patient, who has X-CGD, suffered from multiple fungal osteomyelitis and recurrent IFD despite undergoing voriconazole prophylaxis. Only one patient developed a lupus-like lesion in the cheek, which resolved after discontinuation of voriconazole prophylaxis. Conclusion: Long-term voriconazole prophylaxis may be an alternative option in CGD patients with IFD in terms of patient compliance, efficacy and side effects.