Chronic granulomatous disease

Background

• Primary immunodeficiency of neutrophils leading to recurrent infections with catalase-positive organisms

Pathophysiology

• Defect in NADPH oxidase
• Therefore unable to generate superoxide radicals required by phagocytes to kill pathogens

Clinical Manifestations

• Recurrent infections with catalase-positive organisms, including Staphylococcus aureus
• Highest-risk group for invasive aspergillosis

Management

• Treat intercurrent infections
• Prophylaxis with:
  • TMP-SMX
  • Itraconazole
  • Possibly also interferon-γ