Chronic Granulomatous Disease Medication

Updated: Jul 29, 2019
  • Author: Roman J Nowicki, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Continuous antifungal therapy is effective in preventing infection by Aspergillus species. [41]

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Antibiotics

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

Trimethoprim and sulfamethoxazole (Bactrim, Septra)

Trimethoprim and sulfamethoxazole (TMP-SMZ) is current standard therapy. It inhibits bacterial growth by inhibiting the synthesis of dihydrofolic acid. The antibacterial activity of TMP-SMZ includes common urinary tract pathogens, except Pseudomonas aeruginosa.

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Antifungal agents

Class Summary

These agents exert a fungicidal effect by altering the permeability of the fungal cell membrane. Their mechanism of action may also involve an alteration of RNA and DNA metabolism or an intracellular accumulation of peroxide that is toxic to the fungal cell.

Itraconazole (Sporanox)

Continuous antifungal therapy is effective in preventing infection by Aspergillus species. Itraconazole is a synthetic triazole antifungal agent that slows fungal cell growth by inhibiting cytochrome P-450–dependent synthesis of ergosterol, a vital component of fungal cell membranes.

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Biologic response modifiers

Class Summary

These agents regulate the immune system by a variety of mechanisms including enhancing activity of macrophages and cytotoxic actions of T lymphocytes.

Interferon gamma - 1b (Actimmune)

Interferon (INF)–gamma 1b reduces the frequency and severity of serious infections associated with chronic granulomatous disease (CGD). Interferons are synthesized by eukaryotic cells in response to viruses and a variety of natural and synthetic stimuli. INF-gamma possesses antiviral, immunomodulatory, and antiproliferative activity. INF-gamma has potent phagocyte-activating effects not seen with other interferon preparations, including generation of toxic oxygen metabolites within phagocytes capable of mediating intracellular killing of microorganisms.

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