Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

active non-life-threatening infection: on first presentation

1st line – empiric broad-spectrum antibiotic therapy

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INITIAL
|
active non-life-threatening infection: on first presentation
1st line – 

empiric broad-spectrum antibiotic therapy

Procedures to aid in identification of the infecting organism, such as surgical biopsy of infected sites or bronchoalveolar lavage, are recommended before treatment if available.

Initial treatment is broad, including coverage of Staphylococcus aureus as well as gram-negative organisms.

Infections include pneumonia, cutaneous and soft tissue infections, adenitis, internal abscesses, and osteomyelitis.[3][16][39][52]

Once the organism is identified, directed therapy may be initiated. Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

meropenem: adults: 1 g every 8 hours

OR

levofloxacin: adults: 750 mg intravenously every 24 hours

OR

ciprofloxacin: adults: 200-400 mg intravenously every 12 hours

OR

moxifloxacin: adults: 400 mg intravenously every 24 hours

OR

chloramphenicol: adults: 50-100 mg/kg/day intravenously given in divided doses every 6 hours

OR

cefotaxime: children: 100-200 mg/kg/day intravenously given in divided doses every 8 hours; adults: 1-2 g intravenously every 6-8 hours

OR

ceftriaxone: children: 50-100 mg/kg/day intravenously given in divided doses every 12-24 hours; adults: 1-2 g intravenously every 24 hours

Plus – broad-spectrum antifungal therapy

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INITIAL
|
active non-life-threatening infection: on first presentation
Plus – 

broad-spectrum antifungal therapy

Treatment recommended for ALL patients in selected patient group

Early broad antifungal coverage is often needed as well, specifically for Aspergillus species.

Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

amphotericin B liposomal: children and adults: 3-5 mg/kg intravenously every 24 hours

OR

voriconazole: children ≥2 years of age: 9 mg/kg intravenously every 12 hours on day 1, followed by 8 mg/kg every 12 hours, maximum 700 mg/day; adults: 6 mg/kg intravenously every 12 hours on day 1, followed by 4 mg/kg every 12 hours

OR

posaconazole: adults: 300 mg orally (delayed-release)/intravenously twice daily on day 1, followed by 300 mg once daily; 200 mg orally (suspension) three times daily

Secondary options

itraconazole: children: 5-10 mg/kg/day orally given in 1-2 divided doses, maximum 400 mg/day; adults: 200-400 mg/day orally given in 1-2 divided doses

More

active life-threatening infection: on first presentation

1st line – empiric broad-spectrum antibiotic therapy

Back
INITIAL
|
active life-threatening infection: on first presentation
1st line – 

empiric broad-spectrum antibiotic therapy

Initial treatment is broad, including coverage of Staphylococcus aureus as well as gram-negative organisms.

Infections include pneumonia, cutaneous and soft tissue infections, adenitis, internal abscesses, and osteomyelitis.[3][16][39][52]

Once the organism is identified, directed therapy may be initiated. Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

meropenem: adults: 1 g every 8 hours

OR

levofloxacin: adults: 750 mg intravenously every 24 hours

OR

ciprofloxacin: adults: 200-400 mg intravenously every 12 hours

OR

moxifloxacin: adults: 400 mg intravenously every 24 hours

OR

chloramphenicol: adults: 50-100 mg/kg/day intravenously given in divided doses every 6 hours

OR

cefotaxime: children: 100-200 mg/kg/day intravenously given in divided doses every 8 hours; adults: 1-2 g intravenously every 6-8 hours

OR

ceftriaxone: children: 50-100 mg/kg/day intravenously given in divided doses every 12-24 hours; adults: 1-2 g intravenously every 24 hours

Plus – broad-spectrum antifungal therapy

Back
INITIAL
|
active life-threatening infection: on first presentation
Plus – 

broad-spectrum antifungal therapy

Treatment recommended for ALL patients in selected patient group

Early broad-spectrum antifungal coverage is needed as well, specifically for Aspergillus species.

Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

amphotericin B liposomal: children and adults: 3-5 mg/kg intravenously every 24 hours

OR

voriconazole: children ≥2 years of age: 9 mg/kg intravenously every 12 hours on day 1, followed by 8 mg/kg every 12 hours, maximum 700 mg/day; adults: 6 mg/kg intravenously every 12 hours on day 1, followed by 4 mg/kg every 12 hours

OR

posaconazole: adults: 300 mg orally (delayed-release)/intravenously twice daily on day 1, followed by 300 mg once daily; 200 mg orally (suspension) three times daily

Secondary options

itraconazole: children: 5-10 mg/kg/day orally given in 1-2 divided doses, maximum 400 mg/day; adults: 200-400 mg/day orally given in 1-2 divided doses

More

Consider – granulocyte transfusion

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INITIAL
|
active life-threatening infection: on first presentation
Consider – 

granulocyte transfusion

Treatment recommended for SOME patients in selected patient group

Granulocyte transfusions may be administered as a "last resort" for life-threatening infections.[61][62][63][64][65][50]

The short-term benefits of providing functional granulocytes should be weighed against the risk from exposure to foreign antigens (e.g., HLA antigens).

Consider – interferon gamma

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INITIAL
|
active life-threatening infection: on first presentation
Consider – 

interferon gamma

Treatment recommended for SOME patients in selected patient group

The use of interferon gamma in the treatment of infections in CGD patients remains poorly studied and controversial, although some experts support its use in severely ill patients in the hope of providing benefit.[41]

Consult specialist for guidance on dose and treatment regimens; dose depends on patient's body surface area.[85]

Primary options

interferon gamma 1b: children and adults: consult specialist for guidance on dose

Consider – surgical or radiological drainage

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INITIAL
|
active life-threatening infection: on first presentation
Consider – 

surgical or radiological drainage

Treatment recommended for SOME patients in selected patient group

Surgical or radiological drainage of infected tissue may be required.

ACUTE

following initial empiric treatment

1st line – continued empiric broad-spectrum antibiotic therapy

Back
ACUTE
|
following initial empiric treatment
|
pyogenic hepatic abscess
1st line – 

continued empiric broad-spectrum antibiotic therapy

Treatment is broad-spectrum antibiotic therapy, including coverage of Staphylococcus aureus as well as gram-negative organisms.

Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

meropenem: adults: 1 g every 8 hours

OR

levofloxacin: adults: 750 mg intravenously every 24 hours

OR

ciprofloxacin: adults: 200-400 mg intravenously every 12 hours

OR

moxifloxacin: adults: 400 mg intravenously every 24 hours

OR

chloramphenicol: adults: 50-100 mg/kg/day intravenously given in divided doses every 6 hours

OR

cefotaxime: children: 100-200 mg/kg/day intravenously given in divided doses every 8 hours; adults: 1-2 g intravenously every 6-8 hours

OR

ceftriaxone: children: 50-100 mg/kg/day intravenously given in divided doses every 12-24 hours; adults: 1-2 g intravenously every 24 hours

Plus – corticosteroid

Back
ACUTE
|
following initial empiric treatment
|
pyogenic hepatic abscess
Plus – 

corticosteroid

Treatment recommended for ALL patients in selected patient group

Hepatic abscesses are treated with antibiotics in conjunction with a corticosteroid.[66][67]

Primary options

methylprednisolone sodium succinate: children and adults: 1 mg/kg/day intravenously, taper slowly after clinical response achieved

Consider – broad-spectrum antifungal therapy

Back
ACUTE
|
following initial empiric treatment
|
pyogenic hepatic abscess
Consider – 

broad-spectrum antifungal therapy

Treatment recommended for SOME patients in selected patient group

Early broad-spectrum antifungal coverage may be needed as well, specifically for Aspergillus species.

Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

amphotericin B liposomal: children and adults: 3-5 mg/kg intravenously every 24 hours

OR

voriconazole: children ≥2 years of age: 9 mg/kg intravenously every 12 hours on day 1, followed by 8 mg/kg every 12 hours, maximum 700 mg/day; adults: 6 mg/kg intravenously every 12 hours on day 1, followed by 4 mg/kg every 12 hours

OR

posaconazole: adults: 300 mg orally (delayed-release)/intravenously twice daily on day 1, followed by 300 mg once daily; 200 mg orally (suspension) three times daily

Secondary options

itraconazole: children: 5-10 mg/kg/day orally given in 1-2 divided doses, maximum 400 mg/day; adults: 200-400 mg/day orally given in 1-2 divided doses

More

Consider – granulocyte injection ± interferon-gamma

Back
ACUTE
|
following initial empiric treatment
|
pyogenic hepatic abscess
Consider – 

granulocyte injection ± interferon-gamma

Treatment recommended for SOME patients in selected patient group

The use of normal donor granulocytes injected into lesions has been reported, as well as systemic granulocyte infusions and interferon-gamma administration.[68]

Consider – surgical incision and drainage

Back
ACUTE
|
following initial empiric treatment
|
pyogenic hepatic abscess
Consider – 

surgical incision and drainage

Treatment recommended for SOME patients in selected patient group

Definitive surgical excision and drainage should be considered in patients not responding to antibiotic and corticosteroid therapy.[50] Samples should be taken in all cases to identify the pathogen and guide treatment. Staphylococcus aureus is typically the etiology.[12] Percutaneous drainage may be helpful.

1st line – corticosteroid

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ACUTE
|
following initial empiric treatment
|
inflammatory enterocolitis
1st line – 

corticosteroid

Can be used to treat granulomatous inflammatory lesions, reducing gastrointestinal symptoms in severe disease and flares.

Primary options

prednisone: children: 1 mg/kg orally once daily initially, taper according to response; adults: 5-60 mg orally once daily, taper according to response

1st line – sulfasalazine

Back
ACUTE
|
following initial empiric treatment
|
inflammatory enterocolitis
1st line – 

sulfasalazine

Aids in reducing colonic inflammation in mild disease.

Other more potent immunosuppressive agents may also be used.

Primary options

sulfasalazine: children: 50 mg/kg/day orally given in divided doses every 6 hours; adults: 500 mg orally four times daily; occasionally higher doses may be required

1st line – continued empiric broad-spectrum antibiotic therapy

Back
ACUTE
|
following initial empiric treatment
|
infectious enterocolitis
1st line – 

continued empiric broad-spectrum antibiotic therapy

Treatment is broad-spectrum, including coverage of common enteric pathogens according to local sensitivities.

Early consultation with physicians experienced in the care of CGD is recommended.

Primary options

ciprofloxacin: adults: 200-400 mg intravenously every 12 hours

OR

ceftriaxone: children: 50-100 mg/kg/day intravenously given in divided doses every 12-24 hours; adults: 1-2 g intravenously every 24 hours

1st line – corticosteroid

Back
ACUTE
|
following initial empiric treatment
|
gastrointestinal or genitourinary obstruction
1st line – 

corticosteroid

Most obstructions of hollow viscera can be managed with corticosteroids.

Primary options

prednisone: children: 1 mg/kg orally once daily initially, taper according to response; adults: 30-60 mg orally once daily, taper according to response

Consider – surgery

Back
ACUTE
|
following initial empiric treatment
|
gastrointestinal or genitourinary obstruction
Consider – 

surgery

Treatment recommended for SOME patients in selected patient group

Surgery is indicated for nonresolving obstruction or severe fistulae.[6][8][11]

ONGOING

following resolution of acute episode

1st line – maintenance antibiotic prophylaxis plus vigilant monitoring for infection

Back
ONGOING
|
following resolution of acute episode
1st line – 

maintenance antibiotic prophylaxis plus vigilant monitoring for infection

In the absence of active infections requiring immediate attention, timely referral to a specialist with experience in the management of patients with CGD is warranted.

Prophylaxis with antibiotics should begin promptly with trimethoprim/sulfamethoxazole or other similar medications effective against Staphylococcus aureus and gram-negative organisms. An additional agent may be required to cover methicillin-resistant S aureus if prevalent.

Primary options

sulfamethoxazole/trimethoprim: children and adults: 5 mg/kg orally once or twice daily

More

Secondary options

ciprofloxacin: adults: 250-500 mg orally twice daily

OR

trimethoprim: adults: 100 mg orally once daily at bedtime

OR

cefuroxime axetil: adults: 250-500 mg orally twice daily

OR

cefixime: adults: 400 mg orally once daily

OR

cefpodoxime proxetil: adults: 100-400 mg orally twice daily

Plus – maintenance antifungal prophylaxis

Back
ONGOING
|
following resolution of acute episode
Plus – 

maintenance antifungal prophylaxis

Treatment recommended for ALL patients in selected patient group

Antifungal prophylaxis with itraconazole has become the standard of care for patients with CGD and is shown to decrease the incidence of fungal infection.[71] Aspergillus infections have been the most common cause of death in patients with CGD, although this is changing with widespread use of antifungal prophylaxis.[3][13][16]

If itraconazole is not tolerated, choose an alternative antifungal prophylactic medication that is effective against Aspergillus species.

Voriconazole is an oral alternative, but it carries the risk of reversible liver damage, photosensitivity, and cutaneous malignancy.[58][59][72]

Posaconazole has been used for salvage therapy in patients, although this restricts treatment options for breakthrough infection.[73]

Serum drug levels may be required in patients on azole antifungals.

Primary options

itraconazole: children: 2.5 mg/kg orally twice daily, maximum 200 mg/dose; adults: 200 mg orally twice daily

OR

voriconazole: children ≥2 years of age: 4 mg/kg orally twice daily, maximum 200 mg/dose; adults: 200 mg orally twice daily

OR

posaconazole: children ≥2 years of age and ≥40 kg body weight and adults: 300 mg orally (delayed-release) twice daily on day 1, followed by 300 mg once daily; children ≥13 years of age and adults: 200 mg orally (suspension) three times daily

Consider – maintenance interferon gamma therapy

Back
ONGOING
|
following resolution of acute episode
Consider – 

maintenance interferon gamma therapy

Treatment recommended for SOME patients in selected patient group

Interferon gamma decreases the incidence of infection, although study results are conflicting.[30][74][75][76][77] Additionally, adverse effects limit usage. The routine prophylaxis of infections with interferon gamma varies widely among specialists.

Fever is a common adverse effect associated with interferon treatment. However, the occurrence of fever in a patient with CGD always warrants medical evaluation.

Consult a specialist for guidance on dose and treatment regimens; dose depends on patient's body surface area.[85]

Primary options

interferon gamma 1b: consult specialist for guidance on dose

Consider – evaluation for allogeneic stem cell transplant

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ONGOING
|
following resolution of acute episode
Consider – 

evaluation for allogeneic stem cell transplant

Treatment recommended for SOME patients in selected patient group

Allogeneic stem cell transplantation is a curative procedure, although it carries its own risks of mortality and morbidity particularly if undertaken later in life. Overall survival is greater than 80%, with the majority of surviving patients achieving cure, particularly if an HLA-matched donor is available.[78][79][80][81][82][83][84]

Transplantation should be considered if a matched sibling donor is available.[50] Additionally, matched unrelated (including cord blood) donors should be considered appropriate sources for stem cells in children.

In adults, very careful consideration must be given to the potential risks and benefits of stem cell transplantation.

Stem cell transplantation should be undertaken in medical centers experienced in transplantation for primary immunodeficiency disorders.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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