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Signs and symptoms of chronic granulomatous disease (CGD)

CGD affects multiple organs

Knowing what to look for can aid in the diagnosis of CGD. CGD affects multiple organ systems, particularly any major viscus organ. Granulomas tend to form at sites of hollow organs, like the bladder or GI tract.1

Most frequent sites of infection and common infectious and inflammatory complications1

Patients also exhibit growth retardation and failure to thrive in childhood and have abnormal wound healing.1

Look for the pathogens that may indicate CGD

Patients with primary immunodeficiencies present frequently with chronic and/or recurrent infections caused by a broad array of pathogens and do so early in life. The large majority of severe infections in patients with CGD in North America are caused by a particular group of organisms, both bacterial and fungal.1-7

Most of these pathogens, including Aspergillus, Nocardia, Serratia, Burkholderia, Klebsiella, and Staphylococcus aureus, are catalase-positive.2,7 In patients with CGD, these catalase-positive pathogens are noteworthy because they can neutralize some of the hydrogen peroxide produced by neutrophils other than those from nicotinamide adenine dinucleotide phosphate (NADPH) oxidase.7

Top pathogens and common presentation associated with CGD1-7

  • Fungal
  • Bacterial
Catalase positive fungus of the Aspergillus species

Aspergillus species

Common presentation: pneumonia, lymphadenitis, osteomyelitis, brain abscess

Catalase positive bacteria Nocardia

Nocardia species

Common presentation: pneumonia, osteomyelitis, brain abscess

Catalase positive bacteria Serratia Marcesens

Serratia marcescens

More common presentation: osteomyelitis, soft tissue infections Less common presentation: pneumonia, sepsis

Catalase positive bacteria Burkoderia Cepacia

Burkholderia (formerly pseudomonas) cepacia complex

Common presentation: pneumonia, sepsis

Klebsiella species

Klebsiella species

Common presentation: pneumonia, skin infections, lymphadenitis

Catalase positive bacteria Staphylococcus Aureus

Staphylococcus aureus

Common presentation: soft tissue infections, lymphadenitis, liver abscess, perirectal abscess, osteomyelitis, pneumonia, sepsis

  • Fungal

    Aspergillus species

    Common presentation: pneumonia, lymphadenitis, osteomyelitis, brain abscess

  • Bacterial

    Nocardia species

    Common presentation: pneumonia, osteomyelitis, brain abscess

    Serratia marcescens

    More common presentation: osteomyelitis, soft tissue infections Less common presentation: pneumonia, sepsis

    Burkholderia (formerly pseudomonas) cepacia complex

    Common presentation: pneumonia, sepsis

    Klebsiella species

    Common presentation: pneumonia, skin infections, lymphadenitis

    Staphylococcus aureus

    Common presentation: soft tissue infections, lymphadenitis, liver abscess, perirectal abscess, osteomyelitis, pneumonia, sepsis

Recognize your VEO-IBD patients who may have CGD

GI inflammation is a common manifestation of CGD.8 Approximately 66% of patients with CGD experience chronic and/or acute GI inflammation throughout their lifetime.8 Similarities with IBD may lead to misdiagnosis of CGD.

Median age of CGD diagnosis icon

The median age of diagnosis for CGD is

2.5 to 3
YEARS1

Chronic Granulomatous Disease inflammation icon

In a large study of 98 patients with CGD, the median age at the first inflammatory gastrointestinal (GI) episode was

2.2
YEARS10

CGD IBD icon

CGD is one of the more common monogenic causes of VEO-IBD.8* In children aged <6 years with IBD, a monogenic defect was detected in approximately

15% to 20%9

Early onset IBD icon

All patients with infantile and VEO-IBD should receive

DIAGNOSTIC TESTING9

Patients may present with diarrhea, abdominal pain, constipation, weight loss, failure to thrive, presence of granulomas, and perianal and liver abscesses.1,8

†In general, the gene defects that have been detected with the highest frequency in patients with VEO-IBD can prompt specific targeted therapies that include: defects that lead to CGD (NADPH complex defects), IL-10R, and XIAP.9

GI, gastrointestinal; IL-10R, interleukin-10 receptor; NADPH, nicotinamide adenine dinucleotide phosphate; VEO-IBD, very early onset inflammatory bowel disease; XIAP, X-linked inhibitor of apoptosis protein.

Stay informed about ACTIMMUNE and CGD
  • 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; 1993-2022. 2. Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-1205.e1-e78. 3. Leiding JW, Malech HL, Holland SM. Chronic granulomatous disease. Clinical Focus on Primary Immunodeficiencies. 2013;15:1-9. 4. 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. 5. Bennett N, Maglione PJ, Wright BL, Zerbe C. Infectious complications in patients with chronic granulomatous disease. J Pediatric Infect Dis Soc. 2018;7(suppl_1):S12-S17. 6. Marciano BE, Spalding C, Fitzgerald A, et al. Common severe infections in chronic granulomatous disease. Clin Infect Dis. 2015;60(8):1176-1183. 7. Bortoletto P, Lyman K, Camacho A, et al. Chronic granulomatous disease: a large, single-center US experience. Pediatr Infect Dis J. 2015;34(10):1110-1114. 8. Alimchandani M, Lai J-P, Aung PP, et al. Gastrointestinal histopathology in chronic granulomatous disease: a study of 87 patients. Am J Surg Pathol. 2013;37(9):1365-1372. 9. Kelsen JR, Sullivan KE, Rabizadeh S, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition position paper on the evaluation and management for patients with very early-onset inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2020;70(3):389-403. 10. 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.

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.