What are the recommended immunization and management strategies for a patient with Congenital Adrenal Hyperplasia (CAH) on chronic corticosteroids (steroids)?

Management of CAH Patients on Chronic Steroids: Immunizations and Other Considerations

Immunization Safety and Timing

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

Inactivated Vaccines (Influenza, Pneumococcal, COVID-19, Tdap, etc.)

• Continue all glucocorticoid therapy without interruption when administering inactivated vaccines 1
• Physiologic replacement doses (hydrocortisone 15-20 mg/m²/day or equivalent) do not suppress immune response to inactivated vaccines 1
• Consider high-dose influenza vaccine for patients ≥65 years, though standard dose is acceptable 1
• No dose adjustments or timing modifications needed for routine inactivated vaccines 1

Live-Attenuated Vaccines (MMR, Varicella, Zoster, Yellow Fever)

Live vaccines are safe and can be administered to CAH patients on maintenance physiologic replacement therapy without holding steroids. 1

The critical threshold for immunosuppression concerns is:

• >20 mg/day prednisone equivalent (or >2 mg/kg/day for patients <10 kg) for >2 weeks 1
• CAH patients typically receive hydrocortisone 15-20 mg/m²/day in divided doses, which equals approximately 10-15 mg prednisone equivalent daily—well below the immunosuppressive threshold 2

Physiologic replacement doses that do NOT contraindicate live vaccines include: 1

• Maintenance physiologic doses (replacement therapy)
• Short-term therapy (<14 days)
• Low-to-moderate doses (<20 mg prednisone equivalent/day)
• Alternate-day treatment with short-acting preparations

Evidence from CAH-Specific Studies

A retrospective study of 82 CAH patients (ages 2-40 years) on chronic physiologic steroid replacement demonstrated: 3

• No increased vaccination risk compared to general population
• Complete vaccination rates: diphtheria (79%), tetanus (85%), polio (78%)
• Live vaccine administration (measles 63%, mumps 50%, rubella 38%) without significant complications
• Only 5/82 patients reported side effects, none definitively attributable to CAH or steroid therapy
• No proven vaccination damage in CAH patients on replacement therapy 3

Stress Dosing During Illness and Vaccination

Routine Vaccinations

Do not increase steroid doses for routine vaccinations in stable CAH patients 1, 4

If Fever or Illness Develops Post-Vaccination

• Double the maintenance dose if fever >38.5°C or significant systemic symptoms develop 5
• Continue doubled dose until fever resolves for 24 hours 5
• Standard maintenance: hydrocortisone 15-20 mg/m²/day in 2-3 divided doses (typically 10 mg morning, 5 mg afternoon, 5 mg evening) 5, 2

Emergency Preparedness

All CAH patients must have: 4, 5

• Emergency injectable hydrocortisone kit (100 mg) for self-administration
• Medical alert bracelet/necklace indicating adrenal insufficiency
• Written sick-day management plan
• Education on recognizing adrenal crisis symptoms (severe vomiting, diarrhea, hypotension, altered mental status)

Specific Vaccination Considerations

COVID-19 Vaccination

• Recommended without steroid dose adjustment for CAH patients on physiologic replacement 1
• If on high-dose steroids (>20 mg prednisone equivalent), ideally taper below this threshold before vaccination if disease control permits 1
• For CAH patients, this is rarely an issue as they receive physiologic, not pharmacologic, doses 2

Pneumococcal Vaccination

• Administer pneumococcal vaccine at least 2 weeks before any elective splenectomy if applicable 1
• Standard ACIP recommendations apply for age-appropriate pneumococcal vaccination 1

Influenza Vaccination

• Annual influenza vaccination strongly recommended 1
• High-dose or adjuvanted formulations may provide superior protection in patients ≥65 years 1
• Continue all steroid therapy during vaccination 1

Monitoring and Follow-Up

Annual Assessment Should Include: 2

• Growth parameters (height, weight, BMI)—glucocorticoid excess causes growth suppression
• Blood pressure—both under- and over-replacement affect BP
• Bone age (in children)—advanced bone age indicates poor control
• Biochemical monitoring: morning 17-hydroxyprogesterone, androstenedione, testosterone, plasma renin activity 6, 2
• Vaccination status review—ensure age-appropriate immunizations are current

Optimizing Glucocorticoid Regimen

Recent evidence supports: 7, 6

• Modified-release hydrocortisone (MRHC) given twice daily provides superior disease control compared to immediate-release formulations
• MRHC reduces morning 17-hydroxyprogesterone more effectively (2.5 vs 10.5 nmol/L, p=0.001) 6
• Lower 17-hydroxyprogesterone reduces its mineralocorticoid receptor antagonism, potentially decreasing fludrocortisone requirements 6

Avoid Dexamethasone for Routine Management

Dexamethasone use is associated with: 8

• Greater insulin resistance despite better androgen suppression
• Increased metabolic complications
• Hydrocortisone or prednisolone are preferred for long-term management 8

Critical Pitfalls to Avoid

Never Delay Vaccination Due to Steroid Concerns

• Physiologic replacement doses do NOT contraindicate any vaccine 1, 3
• Delaying vaccination increases risk of vaccine-preventable disease, which poses greater danger than theoretical vaccine risks 1

Never Withhold Steroids During Acute Illness

• Adrenal crisis has high mortality if untreated—always err on the side of stress dosing during significant illness 4, 9
• Vomiting/diarrhea preventing oral intake requires immediate IV hydrocortisone 100 mg bolus 4, 9

Never Start Other Hormone Replacements Before Corticosteroids

• Thyroid hormone, testosterone, or estrogen replacement accelerates cortisol clearance and can precipitate adrenal crisis 5
• Always establish adequate glucocorticoid replacement first 5

Recognize Relative Adrenal Insufficiency

• Even "normal" cortisol levels may be inadequate during physiologic stress 5
• Unexplained hypotension despite fluids/vasopressors warrants empiric hydrocortisone 100 mg IV 5

Practical Algorithm for Vaccination in CAH Patients

Step 1: Verify current steroid dose

• If ≤20 mg prednisone equivalent/day (typical for CAH): proceed with any vaccine 1
• If >20 mg/day for >2 weeks: defer live vaccines until dose reduced or wait 1 month after discontinuation 1

Step 2: Administer vaccine

• Continue all maintenance steroids 1
• No pre-medication needed 1

Step 3: Post-vaccination monitoring

• Routine observation period (15 minutes) 1
• If fever >38.5°C develops: double maintenance dose until afebrile 24 hours 5
• If vomiting/unable to take oral steroids: seek emergency care for IV hydrocortisone 4, 9

Step 4: Document and plan

• Record vaccine in patient chart and personal health record 1
• Ensure patient has emergency hydrocortisone kit and knows when to use it 4, 5