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Examples of prophylactic antimicrobial regimens used in patients with inborn errors of immunity

Examples of prophylactic antimicrobial regimens used in patients with inborn errors of immunity
Infection to be prevented First-line regimen Alternative regimens
Pneumocystis jirovecii Sulfamethoxazole-trimethoprim:
  • Infants >4 weeks of age and children: 5 mg/kg/day orally in 2 divided doses, 3 days/week (based on TMP; maximum 160 mg per dose, 320 mg per day)
  • Adults and adolescents with normal kidney function: 160 mg (based on TMP) daily or 3 days/week, or 80 mg (based on TMP) daily
 Dapsone:
  • Infants and children: 2 mg/kg/dose orally once daily (maximum daily dose: 100 mg/day)
  • Adults: 100 mg once daily or 50 mg twice daily

Atovaquone:

  • 1 to 3 months: 30 mg/kg orally once daily
  • 4 to 24 months: 45 mg/kg orally once daily
  • >24 months: 30 mg/kg orally once daily
  • Adolescents ≥13 years and adults: 1500 mg orally once daily

Pentamidine:

  • Children <5 years: 9 mg/kg (maximum dose: 300 mg/dose) inhalation per nebulizer once every 4 weeks
  • Children >5 years, adolescents and adults: 300 mg inhalation per nebulizer once every 4 weeks
Staphylococcus spp, gram-negative spp Sulfamethoxazole-trimethoprim:
  • Infants >4 weeks of age and children: 5 mg/kg/day orally in 2 divided doses (based on TMP; maximum 160 mg per dose, 320 mg per day)
  • Adolescents and adults: 160 mg (based on TMP) daily or twice daily
 Amoxicillin:*
  • Children: 10 to 20 mg/kg per day as a single dose or divided twice daily (maximum dose 875 mg per day)
  • Adolescents and adults: 875 mg twice daily

Ciprofloxacin:*¶

  • Children: 10 mg/kg/dose twice daily (maximum dose 500 mg)
  • Adults: 500 mg twice daily

Amoxicillin-clavulanate:*

  • Children: 20 mg/kg per day as a single dose or divided twice daily (maximum dose 875 mg per day, based on amoxicillin)
  • Adolescents and adults: 875 mg daily (based on amoxicillin)
Mycoplasma spp, Streptococcus spp, Haemophilus influenzae Azithromycin:
  • Children: 5 to 10 mg/kg/dose orally 3 times weekly (maximum dose of 250 mg)
  • Adolescents and adults: 250 mg orally 3 times weekly
 Amoxicillin:
  • Children: 20 mg/kg/day as a single dose or divided twice daily (maximum dose 875 mg per day)
  • Adolescents and adults: 875 mg orally daily
Nontuberculous mycobacteria Azithromycin:
  • Children: 20 mg/kg/dose orally once weekly (maximum dose 1200 mg weekly but may be given as 600 mg twice per week if higher doses cause nausea)
  • Adolescents and adults: 1200 mg weekly but may be given as 600 mg twice per week if higher doses cause nausea
  
Aspergillus spp Itraconazole:
  • Children: 5 mg/kg/day orally daily (maximum dose 200 mg)
  • Adolescents and adults: 200 mg orally daily
 Posaconazole:Δ
  • <13 years and <34 kg: 6 mg/kg orally 3 times daily immediate-release suspension (maximum dose 200 mg)
  • ≥13 years or 34 to 40 kg: 200 mg immediate-release suspension orally 3 times daily or 200 mg delayed-release tablet daily
  • >40 kg: 300 mg delayed-release tablet daily
Candida spp Fluconazole:
  • Children: 6 mg/kg orally daily (maximum dose 400 mg)
  • Adolescents and adults: 400 mg orally once daily
  
HSV/VZV Acyclovir:◊
  • Children <40 kg: 600 mg/m2/dose or 20 mg/kg/dose orally 4 times per day (maximum 800 mg per dose)
  • Children and adolescents ≥40 kg: 800 mg orally 4 times per day
  • Adults: 800 mg orally twice daily
  
CMV Valganciclovir:
  • Children 1 month to 16 years old: once daily oral dose (mg) = 7 × body surface area × creatinine clearance
  • Adolescents ≥17 years and adults with normal kidney function: 900 mg orally once daily
  
The approach to antimicrobial prophylaxis in patients with various forms of inborn errors of immunity (also known as primary immunodeficiencies) is not standardized. The agents and doses shown in the table are commonly used, but other regimens may also be appropriate. Doses listed in this table are for patients with normal kidney and liver function; several agents require dose adjustment in the setting of kidney or liver impairment.

CMV: cytomegalovirus; HSV: herpes simplex virus; TMP: trimethoprim; VZV: varicella zoster virus.

* No standard second line as varying strains with varying resistance patterns.

¶ Clinicians should weigh the risks of adverse musculoskeletal effects against the benefits when considering long-term use of fluoroquinolones in children.

Δ Requires drug level monitoring.

◊ In patients with overweight or obesity, the weight-based dose of acyclovir is based on ideal body weight (refer to calculator available in UpToDate).

References:
  1. Kuruvilla M, de la Morena MT. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract 2013; 1:573.
  2. Milito C, Pulvirenti F, Cinetto F, et al. Double-blind, placebo-controlled, randomized trial on low-dose azithromycin prophylaxis in patients with primary antibody deficiencies. J Allergy Clin Immunol 2019; 144:584.
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