Should primary care initiate treatment for an adult patient with primary adrenal insufficiency, severe hypocortisolism, and low DHEA (Dehydroepiandrosterone) level, or should treatment be deferred to endocrinology?

Primary Care Can and Should Initiate Treatment for Primary Adrenal Insufficiency

Primary care physicians should initiate treatment immediately for patients with confirmed primary adrenal insufficiency rather than deferring to endocrinology, while arranging early endocrine consultation for ongoing management and patient education. Treatment should never be delayed for specialist referral when the diagnosis is established, as untreated adrenal insufficiency is potentially lethal 1.

Rationale for Primary Care Initiation

Immediate Treatment Imperative

• Treatment of adrenal insufficiency must begin as soon as the diagnosis is confirmed, regardless of setting 1. Delaying therapy while awaiting specialist consultation increases morbidity and mortality risk 2, 3, 4.
• Primary adrenal insufficiency is a life-threatening condition that requires prompt glucocorticoid and mineralocorticoid replacement 5, 6, 7.

Primary Care Capability

The initial management of stable primary adrenal insufficiency is straightforward and well within primary care scope:

Glucocorticoid Replacement:

• Start hydrocortisone 15-25 mg daily in divided doses (typically 2/3 in morning, 1/3 in early afternoon) 1.
• Alternative: prednisone 3-5 mg daily, though hydrocortisone is preferred as it recreates diurnal cortisol rhythm 1, 5.
• Use the lowest dose compatible with patient well-being 1.

Mineralocorticoid Replacement:

• Initiate fludrocortisone 0.05-0.1 mg daily as a single morning dose 1, 8.
• Titrate to achieve normotension, normokalemia, and plasma renin in upper half of reference range 1.
• Typical maintenance range is 50-200 mcg daily 1.

When Endocrine Consultation is Essential

While primary care can initiate therapy, early endocrinology referral should be arranged concurrently for the following critical reasons 1, 2, 3:

Patient Education Requirements

• Teaching stress-dosing protocols (doubling or tripling oral doses during minor illness) 2, 3, 4.
• Training on emergency injectable hydrocortisone use (100 mg IM for severe illness/vomiting) 2, 3, 4, 5.
• Providing medical alert jewelry and emergency steroid cards 1, 2.
• Explaining when to seek immediate medical attention for impending adrenal crisis 1, 2, 3.

Complex Management Scenarios

• Pre-surgical planning requiring stress-dose steroids 1.
• Pregnancy management (dose adjustments in third trimester, IV hydrocortisone during delivery) 1.
• Optimization of replacement therapy when symptoms persist despite treatment 9, 10.

DHEA Replacement Considerations

Regarding the low DHEA level mentioned in your patient:

• There is insufficient evidence to recommend routine DHEA replacement in all patients with primary adrenal insufficiency 1.
• DHEA replacement (typically 25-50 mg daily) may be considered in women with persistent low libido and/or fatigue despite adequate glucocorticoid and mineralocorticoid replacement 1, 6.
• This decision is best made in consultation with endocrinology after optimizing standard hormone replacement 1.

Critical Patient Safety Measures Primary Care Must Implement

Immediate Actions

1. Prescribe emergency injectable hydrocortisone 100 mg with clear instructions for use 2, 3, 4, 5.
2. Provide written stress-dosing guidelines 2, 3, 4.
3. Order medical alert identification 1.
4. Educate on salt intake (encourage liberal salt consumption, avoid licorice and grapefruit juice) 1.

Monitoring Parameters

• Blood pressure (both sitting and standing to detect orthostatic hypotension) 4.
• Serum electrolytes (sodium, potassium) 1.
• Weight and symptoms of over/under-replacement 1.
• Plasma renin activity (target upper half of normal range) 1.

Common Pitfalls to Avoid

Never start thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies, as this can precipitate adrenal crisis 1, 4.

Do not use long-acting steroids like dexamethasone for maintenance therapy in primary adrenal insufficiency, as it lacks mineralocorticoid activity and cannot be easily adjusted 4.

Recognize that even mild gastrointestinal illness can precipitate crisis when patients cannot absorb oral medications 4. Patients must use parenteral hydrocortisone during vomiting/diarrhea 2, 3, 4.

Avoid under-replacement with fludrocortisone, which may manifest as persistent salt craving, postural dizziness, or need for excessive glucocorticoid doses 10.

Severe Hypocortisolism Caveat

If your patient has severe symptomatic hypocortisolism (Grade 2-4: moderate to severe symptoms, hypotension, inability to perform activities of daily living):

• Consider initiating stress-dose hydrocortisone 30-50 mg total daily (or 2-3 times maintenance) 1.
• Assess need for hydration, supportive care, or hospitalization 1, 2, 3.
• Taper to maintenance doses after 2 days once stabilized 1.
• Arrange urgent (not just early) endocrine consultation 1, 2, 3.

In summary: Start treatment now in primary care, refer to endocrinology concurrently for comprehensive education and long-term optimization, but never delay life-saving hormone replacement waiting for a specialist appointment.