Chronic granulomatous disease
ICD-10 D71 ICD-11 4A00.0Y

Treatment of Chronic Granulomatous Disease in Febrile Illness (Fever ≥38°C) or Acute Infection

In patients with chronic granulomatous disease (CGD), any febrile episode or acute infection is a clinical urgency. The underlying immune defect in CGD means infections can escalate rapidly, and prompt, appropriately targeted antibiotic therapy is essential.

Clinical scenario

Febrile illness with temperature of 38°C or above, or acute infection, arising in a patient with confirmed CGD. Any febrile illness must be treated promptly with antibiotics, with escalation to intravenous therapy where clinically necessary.

Treatment approach — partial overview

For severe sepsis in CGD, intravenous combination antibiotic therapy is a key first-line intervention, with agent selection and coverage adapted according to the suspected site of infection and any pathogen subsequently isolated. The complete regimen and decision pathway are available in the full protocol.

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References
  1. Any febrile illness should be treated promptly with antibiotics, proceeding to appropriate intravenous therapy where necessary.
  2. IV Teicoplanin and Ciprofloxacin are a good choice for first line therapy in severe sepsis, with Metronidazole being added if infection below the diaphragm is suspected.
  3. If Staphylococcus is isolated, Flucloxacillin and Fucidin (or another antistaphylococcal antibiotic, e.g. Clindamycin and Clarithromycin) may be used.
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