Management of Congenital Adrenal Hyperplasia (CAH) Patients on Chronic Steroids

Immunization Considerations

The Centers for Disease Control and Prevention (CDC) recommends that patients with CAH on physiologic replacement doses of corticosteroids can safely receive all standard vaccinations, including live-attenuated vaccines, without interruption of their steroid therapy 1, 2, 3
The American Academy of Pediatrics suggests continuing all glucocorticoid therapy without interruption when administering inactivated vaccines 4
Physiologic replacement doses of hydrocortisone (15-20 mg/m²/day or equivalent) do not suppress immune response to inactivated vaccines, according to the CDC 1, 2
Consider high-dose influenza vaccine for patients ≥65 years, though standard dose is acceptable, as recommended by the American College of Rheumatology 4

Live vaccines are safe and can be administered to CAH patients on maintenance physiologic replacement therapy without holding steroids, as stated by the CDC 1, 2, 3
The critical threshold for immunosuppression concerns is >20 mg/day prednisone equivalent (or >2 mg/kg/day for patients <10 kg) for >2 weeks, according to the CDC 1, 2, 3

Do not increase steroid doses for routine vaccinations in stable CAH patients, as recommended by the CDC and the American Academy of Pediatrics 1, 5
Double the maintenance dose if fever >38.5°C or significant systemic symptoms develop, as suggested by the American Academy of Pediatrics 6

COVID-19 vaccination is recommended without steroid dose adjustment for CAH patients on physiologic replacement, according to the Infectious Diseases Society of America 7, 8
Pneumococcal vaccine should be administered at least 2 weeks before any elective splenectomy, as recommended by the CDC 1
Annual influenza vaccination is strongly recommended, with high-dose or adjuvanted formulations providing superior protection in patients ≥65 years, according to the American College of Rheumatology 4

Annual assessment should include growth parameters, blood pressure, and bone age, as recommended by the American Academy of Pediatrics 5
Vaccination status review should ensure age-appropriate immunizations are current, according to the CDC 8

Verify current steroid dose and proceed with vaccination if ≤20 mg prednisone equivalent/day, as recommended by the CDC 1, 2
Administer vaccine and continue all maintenance steroids, with no pre-medication needed, according to the CDC 1, 4
Post-vaccination monitoring should include routine observation period and doubling maintenance dose if fever >38.5°C develops, as suggested by the American Academy of Pediatrics 6

The Centers for Disease Control and Prevention recommends that live vaccines be withheld in patients receiving prednisone ≥20 mg/day (or equivalent) for ≥14 days 9, 10, 11
The Centers for Disease Control and Prevention recommends that live vaccines be withheld in patients receiving ≥2 mg/kg/day for patients weighing >10 kg for ≥14 days 9, 10, 12
After discontinuation of high-dose therapy, the Centers for Disease Control and Prevention recommends waiting at least 1 month before administering live vaccines 9, 10, 12
Some experts recommend waiting 3 months for maximum safety 11

The following live vaccines are contraindicated during high-dose immunosuppressive steroid therapy: MMR (measles, mumps, rubella), Varicella (chickenpox), live attenuated influenza (intranasal), Yellow fever, Oral typhoid (Ty21a), BCG, Live zoster vaccine (Zostavax), and Rotavirus 11
The American Academy of Pediatrics and the Centers for Disease Control and Prevention note that recombinant zoster vaccine (Shingrix) is NOT a live vaccine and can be given to patients on any dose of steroids 11

The Centers for Disease Control and Prevention states that all inactivated vaccines can be administered regardless of steroid dose, including Hepatitis vaccines 11 and Tdap/Td 9

The Centers for Disease Control and Prevention recommends determining current prednisone dose (or equivalent) and duration, and if ≥20 mg/day for ≥14 days, live vaccines are contraindicated 9, 10
The Centers for Disease Control and Prevention recommends that if live vaccine is contraindicated, determining urgency, and if not urgent, waiting until dose tapered below 20 mg/day, then waiting additional 1 month 9, 10
The American Academy of Pediatrics recommends that infants exposed to biologics in utero avoid live vaccines for 12 months (except rotavirus may be given with anti-TNF exposure) 11
The Centers for Disease Control and Prevention recommends that patients on combination immunosuppression apply same thresholds but exercise greater caution 9, 11