Addisons Disease (AD) – 49 Symptoms and Signs, Prevalence, Prognosis, Treatment

What is Addisons Disease (AD)

Addisons Disease is a rare endocrine disorder. Usually this condition is chronic i nature. This disorder is due to autoimmune destruction of a structure in a gland in the body known as adrenal gland, this structure is the adrenal cortex.

This process leads to deficiency of certain hormones called glucocorticoid and mineralocorticoid.

Addisons Disease is also termed as the Autoimmune adrenalitis, but this term is usually to describe any form of a condition called chronic primary adrenal insufficiency (CPAI).

Synonyms

  • Autoimmune Addisons disease
  • Autoimmune adrenalitis
  • Classic Addisons disease
  • Primary Addisons disease

Prevalence

  • 1-5 / 10 000
  • 1/9,000-1/6,900 in the developed countries

Age of onset

  • All ages
  • The peak age of the onset of the disease is around 40 but it can occur at any age

Etiology

The etiology of Addisons Disease is of autoimmune origin. AD is due the autoimmune destruction of the adrenal cortex.

Mandatory Symptoms (100% present)

  • Hypocortisolemia 
  • Primary adrenal insufficiency 

Very Common Symptoms (80%-98%)

  • Abdominal pain 
  • Anorexia 
  • Autoimmunity 
  • Constipation 
  • Diarrhea 
  • Failure to thrive 
  • Fatigue 
  • Hyperpigmentation of the skin 
  • Hypotension 
  • Increased circulating ACTH level 
  • Muscle weakness 
  • Nausea and vomiting 
  • Weight loss 

Common Symptoms (30%-79%)

  • Androgen insufficiency 
  • Decreased circulating aldosterone level 
  • Decreased urinary potassium 
  • Hyperkalemia 
  • Hyperkalemic metabolic acidosis 
  • Hyperuricemia 
  • Hyponatremia 
  • Increased circulating renin level 
  • Normocytic anemia 
  • Renal salt wasting

Occasional Symptoms (5%-29%)

  • Adrenal calcification 
  • Adrenal hypoplasia 
  • Arthralgia 
  • Celiac disease 
  • Decreased female libido 
  • Delayed puberty 
  • Dry skin 
  • Generalized bone demineralization 
  • Hashimoto thyroiditis 
  • Hypercalcemia 
  • Hypoglycemia 
  • Hypoparathyroidism 
  • Orthostatic hypotension 
  • Premature ovarian insufficiency 
  • Primary ovarian failure 
  • Salt craving 
  • Seizures 
  • Sparse axillary hair 
  • Type I diabetes mellitus 
  • Vertigo 
  • Vitiligo 

Rare Symptoms (4%-1%)

  • Primary testicular failure 
  • Thiamine responsive megaloblastic anemia 
  • Thymoma

Prognosis

  • Unfortunately, there is no cure for AD.
  • The good news is the prognosis is good with proper treatment, precautions and care.
  • Normally life expectancy is not reduced.
  • Addisons Disease is only life threatening when ignored.

Treatment of Addisons Disease

  • Usually the Management of AD is life long and requires a multidisciplinary approach.
  • Oral hydrocortisone with doses of 10-25 mg daily in 2-3 doses. This Glucocorticoid replacement is given to help mimic the physiological cortisol pattern of secretion in the body.
  • Normally, Oral fludrocortisone may be given to replace mineralocorticoid hormones.
  • Another optional treatment is the replacement of Dehydroepiandrosterone.
  • The levels of Glucocorticoids doses can be adjusted during times of stress.
  • How to monitor an maintain the doses of hydrocortisone – The best method is to assess the requirements of the doses based on the clinical examination of the affected, also taking into consideration the responses from the individual, and their well-being. Additionally, checking any presence of signs of over-replacement or under-replacement.
  • Detection of the activity of a compound in the body known as plasma renin is very beneficial to optimize the dose of fludrocortisone.
  • Regular monitoring of the Growth and development in children is mandatory.
  • In case of suspected adrenal crisis, it is advisable for the affected to carry with them a ready to inject hormone called hydrocortisone preparation and also carry a medical alert card.
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