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Symptoms and Treatment of Adrenal Insufficiency and Addisonian Crisis

Definition

Adrenal insufficiency is hypocortisolism due to dysfunction of the adrenal cortex, hypophysis or hypothalamus with a potentially fatal outcome (Arlt et al., 2003).

Epidemiology

Prevalence 1/40.000, Mortality 0,3–1/100.000.

Etiology

• Diseases of the adrenals (Morbus Addison): after (bilateral) adrenalectomy, autoimmune disease, tuberculosis, adrenal metastases, sepsis. Medications: ketoconazole, mitotane, suramin. Rare causes are sarcoidosis, infections such as histoplasmosis, blastomycosis, AIDS.
• Diseases of hypophysis and hypothalamus:
  • Acute withdrawal from steroids (discontinuation of medication, adrenalectomy).
  • Pituitary and hypothalamic diseases: Tumors, sarcoidosis, tuberculosis, radiation, infarcts.
  • Sheehan syndrome: ischemic necrosis of the hypophysis due to heavy postpartum bleeding and shock.

Signs and Symptoms

• Acute adrenal crisis: clinical deterioration, fever, vomiting, abdominal pain, hypotension, lethargy. Synonyms: Addisonian crisis.
• Chronic adrenal insufficiency: fatigue, weight loss, nausea and vomiting, hyperpigmentation of the skin and mucous membranes, abdominal discomfort, diarrhea, salt starvation, hypotension.

Diagnosis of Adrenal Insufficiency

• Laboratory tests: hyponatremia, hyperkalemia, hypoglycemia. Urine cortisol decreased, serum cortisol decreased, serum ACTH increased or decreased depending on cause. Determination of autoantibodies against the adrenal cortex.
• ACTH test: inadequate increase in serum cortisol 60 min after administration of ACTH (0.25 mg i.v.).
• CRH test: inadequate increase of ACTH and cortisol after administration of CRH (1 μg/kgKG i.v.).
• Imaging: MRI or CT of the head or abdomen, depending on the suspected diagnosis.

Treatment of Adrenal Insufficiency

Emergency Treatment of Addisonian crisis:

• Rapid infusion of 0.9% NaCl (2–3 l).
• Administration of 100 mg hydrocortisone i.v. as a bolus, continuous infusion of another 100 mg hydrocortisone over 24 hours.
• Treat hyponatremia.
• After stabilization of the patient, a short ACTH test is necessary to confirm the diagnosis.

Perioperative Substitution Therapy after Bilateral Adrenalectomy:

• OP day: hydrocortisone 100 mg i.v. during anesthesia induction, then hydrocortisone perfusor 100 mg/24 h i.v.
• Day 1–3: hydrocortisone 100 mg/24 h i.v.
• Day 4: hydrocortisone 20-20-10 mg p.o.
• Following days: in case of uncomplicated course reduction by 10 mg/d until maintenance dose (hydrocortisone 20-10-0 mg/d) is reached, if hydrocortisone is below 50 mg/d, fludrocortisone 0.1 mg/d is additionally necessary.
• Control of blood pressure, electrolytes and renin activity.

Substitution Therapy for Chronic Adrenal Insufficiency:

• Hydrocortisone 20-10-0 mg/d
• Fludrocortisone 0.1 mg/d
• Dosage increase is important in case of infections or surgery.
• Control of blood pressure, electrolytes and renin activity.
• Issuance of an steroid emergency card.

References

Arlt und Allolio 2003 ARLT, W. ; ALLOLIO, B.: Adrenal insufficiency.
In: Lancet
361 (2003), Nr. 9372, S. 1881–93

  Deutsche Version: Akute Addison Krise und Therapie der Nebenniereninsuffizienz