Initiate Glucocorticoid Therapy for All Confirmed PAI Patients
STEP 1 — Gather Information
Collect morning cortisol, plasma ACTH, and perform a 250 µg ACTH stimulation test; assess electrolytes, symptoms (fatigue, hypotension, hyperpigmentation), and review for mineralocorticoid deficiency.
Action: Confirm PAI diagnosis via subnormal cortisol response (<500 nmol/L) to 250 µg ACTH or elevated ACTH with low cortisol.
STEP 2 — Rule In / Rule Out
Is PAI confirmed by biochemical testing?
- Yes: Proceed to Step 3.
- No: Consider alternative diagnoses; do not initiate glucocorticoid therapy for PAI.
STEP 3 — Classify or Stratify
Classify patient as adult or pediatric to determine appropriate glucocorticoid dosing regimen.
Action: Select adult or pediatric dosing pathway.
STEP 4 — Decide
Initiate glucocorticoid replacement: adults hydrocortisone 15–25 mg/day or cortisone acetate 20–35 mg/day in two to three divided doses; children hydrocortisone 8 mg/m²/day in three to four divided doses. Add fludrocortisone if mineralocorticoid deficient, educate on stress dosing, and provide emergency steroid card.
Action: Prescribe replacement regimen and arrange follow‑up.
Clinical Guardrails / Mimics / Pitfalls
Do not use dexamethasone due to risk of Cushingoid side effects; avoid relying solely on hormonal levels to adjust dose—use clinical assessment (weight, blood pressure, energy). Ensure mineralocorticoid replacement when needed and provide steroid emergency card with clear stress‑dosing instructions.
Concrete Clinical Example
A 50‑year‑old man with confirmed PAI (ACTH stimulation test peak cortisol 300 nmol/L, ACTH nmol/L, ACTH 150 pmol/L) started on hydrocortisone 20 mg daily (10 mg on waking, 5 mg early afternoon, 5 mg late afternoon) plus fludrocortisone 100 µg daily; received steroid card and instructed to double dose during fever >38 °C.
Source: Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline, Endocrine Society, 2016, DOI:10.1210/jc.2015-1710