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X-linked chronic granulomatous disease (CGD)

Understanding the risk of X-linked patients

X-linked CGD accounts for approximately 65% of CGD cases.1 About 10%-15% of X-linked mutations are the result of new random mutations.2
X-linked CGD often presents early in life with most cases diagnosed by age 5.3

X-linked carriers may experience infections and other symptoms

X-linked carriers can experience various symptoms and may be at risk for serious infections.* Symptomatic mothers of sons with X-linked CGD should be monitored as potential patients as they may present with signs of CGD and experience a wide range of inflammatory and autoimmune symptoms, including4-8

A 2018 study found that 48% of X-linked carriers reported infections, autoimmune symptoms, or both.5 As a result, X-linked carriers of CGD may be at risk of serious infection, should be evaluated, routinely tested, and monitored as patients, as DHR values may change over time.5

The importance of testing X-linked carriers

Carrier testing should be done for at-risk relatives of patients with CGD, especially for females, because autoimmune events are more common in female X-linked carriers than in healthy individuals.3,6 Female X-linked carriers can demonstrate both normal and deficient neutrophil function.7,8

Genetic testing should be performed on females to determine if they are at risk of passing CGD on to subsequent family members or passing on the trait.

The dihydrorhodamine (DHR) test, a common test for CGD, has the ability to assess neutrophil superoxide production. Based on testing, carriers of X-linked CGD may require prophylactic medical management that can change over time as age-related skewing of lyonization may occur as the carriers age. Healthcare providers should monitor carriers and periodically obtain follow-up DHR testing as neutrophil function can decline over time, thereby increasing the risk of potentially serious or life-threatening infections.9

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

Stay informed about ACTIMMUNE and CGD
  • References

    1. Noack D, Rae J, Cross AR, et al. Autosomal recessive chronic granulomatous disease caused by defects in NCF-1, the gene encoding the phagocyte p47-phox: mutations not arising in the NCF-1 pseudogenes. Blood. 2001;97(1):305-311. 2. Rider NL, Jameson MB, Creech CB. Chronic granulomatous disease: epidemiology, pathophysiology, and genetic basis of disease. J Pediatric Infect Dis Soc. 2018;7(suppl 1):S2-S5. 3. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Adam MP, Ardinger HH, et al., eds. GeneReviews® Washington, Seattle; 1993-2022. 4. Battersby AC, Bruggins H, Pearce MS, et al. Inflammatory and autoimmune manifestations in X-linked carriers of chronic granulomatous disease in the United Kingdom. J Allergy Clin Immunol. 2017;140(2):628-630.e6. 5. Marciano BE, Zerbe CS, Falcone EL, et al. X-linked carriers of chronic granulomatous disease: Illness, lyonization, and stability. J Allergy Clin Immunol. 2018;141(1):365-371. 6. Magnani A, Brosselin P, Beauté J, et al. Inflammatory manifestations in a single-center cohort of patients with chronic granulomatous disease. J Allergy Clin Immunol. 2014;134(3):655-662.e8. 7. Song E, Jaishankar GB, Saleh H, Jithpratuck W, Sahni R, Krishnaswamy G. Chronic granulomatous disease: a review of the infectious and inflammatory complications. Clin Mol Allergy. 2011;9(1):10-24. 8. Holland SM. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38(1):3-10. 9. Holland SM. Chronic granulomatous disease. Hematol Oncol Clin North Am. 2013;27(1):89-99.

Approved Uses and 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-1b, 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
    • Myelosuppression or receiving other potentially myelosuppressive agents
    • Severe renal insufficiency
    • Age <1 year
  • Monitoring:
    • Before starting ACTIMMUNE and every 3 months during treatment, hematologic tests, blood chemistries, and urinalysis are recommended for all patients
    • Patients begun on ACTIMMUNE before the age of 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

USE IN SPECIFIC POPULATIONS

  • ACTIMMUNE should be used during pregnancy only if the potential benefit to the patient outweighs the potential risk to the fetus
  • It is not known if ACTIMMUNE is excreted in human milk, so either ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the patient
  • In younger patients, long-term effects of ACTIMMUNE on fertility are not known
  • In animal studies, both male and female fertility was negatively impacted by doses significantly higher than the maximum clinical dose

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

Please see Full Prescribing Information for additional safety information.

Approved Uses and 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-1b, 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
    • Myelosuppression or receiving other potentially myelosuppressive agents
    • Severe renal insufficiency
    • Age 1 year
  • Monitoring:
    • Before starting ACTIMMUNE and every 3 months during treatment, hematologic tests, blood chemistries, and urinalysis are recommended for all patients
    • Patients begun on ACTIMMUNE before the age of 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

USE IN SPECIFIC POPULATIONS

  • ACTIMMUNE should be used during pregnancy only if the potential benefit to the patient outweighs the potential risk to the fetus
  • It is not known if ACTIMMUNE is excreted in human milk, so either ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the patient
  • In younger patients, long-term effects of ACTIMMUNE on fertility are not known
  • In animal studies, both male and female fertility was negatively impacted by doses significantly higher than the maximum clinical dose

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

Please see Full Prescribing Information for additional safety information.