This page is for healthcare professionals treating patients with chronic granulomatous disease.
This page is for HCPs treating patients with CGD.

NADPH oxidase: the biochemical basis of CGD

See how a mutation in any of the 5 nicotinamide adenine dinucleotide phosphate (NADPH) oxidase component genes can lead to life-threatening bacterial and fungal infections.

Watch: NADPH oxidase

Chronic granulomatous disease (CGD), a rare genetic disorder that benefits from early detection

Chronic granulomatous disease (CGD) is a rare inherited primary immunodeficiency disorder that affects about 1 in 200,000 people in the United States. This primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, and macrophages) results from impaired killing of fungi and bacteria, which can lead to severe and recurrent infections.1,2

Early diagnosis and benefits of treatment

Early diagnosis and prophylactic treatment is beneficial for patients with CGD because they are more susceptible to and have a higher incidence of serious infections.*,1,3 The recommended treatment paradigm consists of an antibacterial, an antifungal, and immunomodulatory therapy with ACTIMMUNE® (Interferon gamma-1b).1,3-5 ACTIMMUNE® is indicated for reducing the frequency and severity of serious infections associated with CGD.6,7

With the development of preventative therapies and the early recognition of infectious complications, 90% of children with CGD now survive into adulthood. This remains, however, largely dependent on several factors, including time of diagnosis, access to care, expertise of caregiver, adherence to therapy, and lifestyle modifications.3,4

*Serious infection is defined as a clinical event requiring hospitalization and intravenous antibiotics.

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References: 1. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993-2016. 2. Winkelstein JA, Marino MC, Johnston RB Jr, et al. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine (Baltimore). 2000;79(3):155-169. 3. Holland SM. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38(1):3-10. 4. Thomsen IP, Smith MA, Holland SM, Creech CB. A comprehensive approach to the management of children and adults with chronic granulomatous disease. J Allergy Clin Immunol Pract. 2016;4(6):1082-1088. 5. Gallin JI, Alling DW, Malech HL, et al. Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med. 2003;348(24):2416-2422. 6. ACTIMMUNE® (Interferon gamma-1b) [prescribing information]. Dublin, Ireland: Horizon Pharma Ireland Ltd; 2016. 7. The International Chronic Granulomatous Disease Cooperative Study Group. A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. N Engl J Med. 1991;324(8):509-516.

Important Safety Information

INDICATIONS AND USAGE

ACTIMMUNE® (Interferon gamma-1b) is indicated:

Important Safety Information

INDICATIONS AND USAGE

ACTIMMUNE® (Interferon gamma-1b) is indicated:

  • For reducing the frequency and severity of serious infections associated with Chronic Granulomatous Disease
  • For delaying time to disease progression in patients with severe, malignant osteopetrosis

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

  • In patients who develop or have known hypersensitivity to interferon-gamma, E coli-derived products, or any component of the product

WARNINGS AND PRECAUTIONS

  • ACTIMMUNE should be used with caution in patients with:
    • Pre-existing cardiac conditions, including ischemia, congestive heart failure, or arrhythmia
    • Seizure disorders or compromised central nervous system function; reduce dose or discontinue
    • Myelosuppression, or receiving other potentially myelosuppressive agents; consider dose reduction or discontinuation of therapy
    • Severe renal insufficiency
    • Age <1 year

  • Monitoring:
    • Patients begun on ACTIMMUNE before age 1 year should receive monthly assessments of liver function. If severe hepatic enzyme elevations develop, ACTIMMUNE dosage should be modified
    • Monitor renal function regularly when administering ACTIMMUNE in patients with severe renal insufficiency; accumulation of interferon gamma-1b may occur with repeated administration. Renal toxicity has been reported in patients receiving ACTIMMUNE

  • Pregnancy, Lactation, and Fertility:
    • ACTIMMUNE should be used during pregnancy only if the potential benefit outweighs the potential risk to the fetus
    • Use of ACTIMMUNE by lactating mothers is not recommended. ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the mother
    • Long-term effects of ACTIMMUNE on fertility are not known

DRUG INTERACTIONS

  • Concomitant use of drugs with neurotoxic, hematotoxic, or cardiotoxic effects may increase the toxicity of interferons
  • Avoid simultaneous administration of ACTIMMUNE with other heterologous serum protein or immunological preparations (eg, vaccines)

ADVERSE REACTIONS

  • The most common adverse experiences occurring with ACTIMMUNE therapy are “flu-like” symptoms such as fever, headache, chills, myalgia, or fatigue, which may decrease in severity as treatment continues, and may be minimized by bedtime administration of ACTIMMUNE. Acetaminophen may be used to prevent or partially alleviate the fever and headache
  • Isolated cases of acute serious hypersensitivity reactions have been observed in patients receiving ACTIMMUNE
  • Reversible neutropenia, thrombocytopenia, and elevations of AST and/or ALT have been observed during ACTIMMUNE therapy
  • At doses 10 times greater than the weekly recommended dose, ACTIMMUNE may exacerbate pre-existing cardiac conditions, or may cause reversible neurological effects such as decreased mental status, gait disturbance, and dizziness