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Addison's Disease Summary

Key Facts

  • Primary adrenal insufficiency
  • Autoimmune most common cause (UK)
  • Tuberculosis worldwide
  • Cortisol & aldosterone deficiency

Clinical Features

  • Fatigue, weight loss
  • Hyperpigmentation
  • Salt cravings
  • Postural hypotension
  • GI symptoms

Key Investigations

  • Serum cortisol
  • Synacthen test
  • ACTH levels
  • U&E (hyponatraemia)
  • Renin/aldosterone

Treatment

  • Hydrocortisone: 15-30mg daily
  • Fludrocortisone: 50-300mcg daily
  • Lifelong replacement
  • Stress dose adjustment

Adrenal Crisis

  • IM Hydrocortisone: 100mg adult dose
  • IV fluids (saline)
  • Emergency management
  • Hospital admission

Addison's Disease (Primary Adrenal Insufficiency)

Addison's disease (primary adrenal insufficiency) is a result of destruction of the adrenal cortex resulting in reduced production of glucocorticoids (such as cortisol), mineralocorticoids (such as aldosterone) and adrenal androgens (such as dehydroepiandrosterone). The absence of cortisol leads to increased production of adrenocorticotropic hormone (ACTH) because negative feedback to the pituitary gland is reduced.

Causes

Clinical Features

Diagnosis of Addison's disease is often delayed because symptoms are non-specific, common, and overlap with many other conditions.

General Symptoms

  • Fatigue
  • Weight loss
  • Muscle weakness
  • Low-grade fever

Characteristic Signs

  • Hyperpigmentation
  • Salt cravings
  • Postural hypotension
  • Loss of axillary/pubic hair

GI Symptoms

  • Nausea and vomiting
  • Abdominal pain
  • Loss of appetite
  • Premature satiety

Adrenal Crisis Warning

A person with Addison's disease may present with a sudden crisis precipitated by intercurrent infection or stress. The adrenal glands cannot supply the extra corticosteroids needed to cope with the stress, and life-threatening symptoms develop. Features include hypotension, hypoglycaemia, acute abdominal pain, low-grade fever, and vomiting. Even if the person is young and otherwise fit, adrenal crisis may result in severe dehydration, hypovolaemic shock, altered consciousness, seizures, stroke, or cardiac arrest.

Investigations

Serum Cortisol

<100 nmol/L: Highly likely adrenal insufficiency

100-500 nmol/L: Refer for Synacthen test

U&E

Hyponatraemia: 90% of cases

Hyperkalaemia: 65% of cases

Synacthen Test

ACTH stimulation test

Normal response: increased cortisol

Management

Routine Hormone Replacement

  • Glucocorticoid: Hydrocortisone 15-30 mg daily in divided doses
  • Mineralocorticoid: Fludrocortisone 50-300 mcg daily
  • Androgen replacement: DHEA not routinely prescribed in UK

Self-care Advice

  • Lifelong glucocorticoid replacement required
  • Adjust medication during illness, injury, or stress
  • Recognize adrenal crisis symptoms
  • Carry emergency information (MedicAlert)
  • Inform healthcare providers about condition

Adrenal Crisis Management

  • Hydrocortisone IM/IV:
    • Adults: 100 mg
    • Children 6+ years: 50-100 mg
    • Children 1-5 years: 50 mg
    • Infants <1 year: 25 mg
  • IV saline infusion for stabilization
  • No emergency fludrocortisone required (high-dose hydrocortisone has mineralocorticoid effect)

Other Adrenal Disorders

Primary Hyperaldosteronism

  • Excess aldosterone production
  • Hypertension, hypokalaemia
  • Aldosterone/renin ratio screening
  • 30% unilateral (surgery)
  • 70% bilateral (medication)

Phaeochromocytoma

  • Catecholamine-producing tumor
  • Headaches, sweating, palpitations
  • Paroxysmal hypertension
  • Urine metanephrines diagnostic
  • Surgical excision treatment

Cushing's Syndrome

  • Pathological hypercortisolism
  • Central obesity, moon face
  • Proximal muscle weakness
  • Metabolic complications
  • Various causes (pituitary, adrenal, ectopic)

Other Causes of Adrenal Insufficiency