Primary Adrenal Insufficiency / Crisis
Clinical Presentation:
- symptoms depend on the rate of decline of function and superimposed illness
- must have a high suspicion as the presentation can be non-specific including nausea, vomiting, fatigue, weakness, fever and confusion
- more specific aspects of the presentation include a history of weight loss, hyperpigmentation (scars, bucal mucosa), less commonly hypoglycemia
other autoimmune diseases may be present
Underlying Etiology of Primary Adrenal Insufficiency
- Autoimmune (Addison’s or autoimmune polyglandular dz especially type II “Schmidts syndrome”)
- Infection: TB, HIV, fungal, CMV
- Cancer/metasteses
- Hemorrhage – Waterhouse-Friedrichsen (GC) or anticoagulants
- Infarct – APLA
- Drugs: ketoconazole, mitotane
- Adrenomyeloneuropathy – X-linked
On Exam:
- hypotension
- reduced BMI
- hyperpigmentation
- hypogonadism – with changes in hair distribution patterns in women
Laboratory Features
- CBC (eosinophilia, lymphocytosis)
- Lytes: hyponatremia from decreased aldosterone (primary) or cortisol/increased ADH (secondary), hyperkalemia, hypercalcemia
- Glucose: hypoglycaemia – more common in secondary due to glucocorticoid deficiency
- Non-anion gap metabolic acidosis
Diagnosis
- Should be made with serum measurements of renin, ACTH and cortisol at baseline
diagnosis is confirmed by a cosyntropin (ACTH) stimulation test.
Treatment
- In a ‘crisis’ needs to be initiated before the diagnosis is confirmed (due to hypotension etc)
- short term : dexamethasone 4-8mg iv bolus can be used as it doesn’t interfere with the stim test assays
- in patients with previous established disease hydrocortisone may be preferred due to mineralocorticoid activity
- long term: glucocorticoid therapy with hydrocortisone, prednisone, dexamethasone + mineralocorticoid therapy with flourinef
- consider: adrogen replacement in select circumstances
- *** counselling re illness, surgery, pregnancy and stress steroids
- ***medical alert bracelet
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