Sources say his tan was from Cape Cod.....

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