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Addison’s disease

Background knowledge ๐Ÿง 

Definition

  • Addison’s disease is a chronic endocrine disorder where the adrenal glands produce insufficient amounts of cortisol and aldosterone
  • Also known as primary adrenal insufficiency
  • Characterized by a gradual onset of symptoms

Epidemiology

  • Incidence: 4-6 per 100,000 per year
  • More common in women than men
  • Can present at any age, but most commonly diagnosed in middle age
  • Increased prevalence in individuals with autoimmune disorders

Aetiology and Pathophysiology

  • Autoimmune destruction of the adrenal cortex (most common cause)
  • Infections (e.g., tuberculosis, HIV)
  • Adrenal hemorrhage or infarction
  • Genetic disorders (e.g., adrenoleukodystrophy)
  • Pathophysiology: loss of adrenal cortex function leads to deficient production of cortisol and aldosterone

Clinical Features ๐ŸŒก๏ธ

Symptoms

  • Fatigue and weakness
  • Weight loss and decreased appetite
  • Hyperpigmentation of the skin
  • Hypotension (postural)
  • Nausea, vomiting, and abdominal pain
  • Salt craving
  • Dizziness and fainting

Signs

  • Hyperpigmentation (especially in creases of hands, gums, and scars)
  • Low blood pressure (especially postural hypotension)
  • Dehydration
  • Loss of body hair (in females)
  • Hypoglycemia
  • Hyponatremia and hyperkalemia

Investigations ๐Ÿงช

Tests

  • Serum cortisol (low) and ACTH (high in primary Addison’s)
  • Short Synacthen test (assesses adrenal response to ACTH)
  • Electrolytes: hyponatremia, hyperkalemia
  • Plasma renin activity and aldosterone levels
  • Autoantibody tests (e.g., anti-adrenal antibodies)
  • Imaging: CT/MRI of adrenal glands if indicated

Management ๐Ÿฅผ

Management

  • Lifelong hormone replacement therapy (hydrocortisone and fludrocortisone)
  • Patient education on stress dose adjustments
  • Emergency hydrocortisone injection kit
  • Regular monitoring of electrolytes and symptoms
  • Treatment of underlying cause if identified (e.g., infection)
  • Medical alert identification (e.g., bracelet)

Complications

  • Adrenal crisis (acute adrenal insufficiency)
  • Electrolyte imbalances
  • Hypoglycemia
  • Osteoporosis (due to long-term corticosteroid use)
  • Increased susceptibility to infections
  • Cardiovascular issues (e.g., hypotension)

Prognosis

  • Good with appropriate treatment and monitoring
  • Requires lifelong hormone replacement
  • Regular follow-up necessary to adjust therapy and prevent complications
  • Patients can lead a normal life with proper management
  • Prognosis depends on timely diagnosis and treatment adherence

Key Points

  • Addison’s disease is a chronic condition requiring lifelong treatment
  • Early recognition and treatment are crucial to prevent adrenal crisis
  • Patient education on medication adherence and stress management is essential
  • Regular follow-up and monitoring improve outcomes
  • Management involves a multidisciplinary approach

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