| Addison's Disease |
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Prepared by: Daniela Cihakova MD, PhD Definition: Autoimmune Addison’s disease is an autoimmune inflammation of adrenal glands, resulting in their gradual destruction and inability to make sufficient amounts of adrenal hormones. Description: Addison’s disease is a disease of the adrenal glands. They are endocrine glands located above the kidneys. They produce two hormones; Cortisol and Aldosterone. Addison’s disease occurs when adrenal glands do not produce enough Cortisol and sometimes Aldosterone. Production of Cortisol is regulated by the pituitary gland's secretion of ACTH (adrenocorticotropin hormone). Symptoms:
Diagnosis: Addison’s disease can be difficult to diagnose. The clearest symptom is a darkening of the skin, but it may not be present in some patients and it is never present in secondary Addison’s disease. Diagnosis is made by assessment of Cortisol levels in blood. Common test used to diagnose Addison's Disease include:
Incidence:
Treatment: Addison’s disease is treated by substituting the missing Cortisol, eventually Aldosterone as well. Both hormones are available as oral tablets. Patient should notify doctors about their diagnosis condition prior to any surgery, or before delivery, since more Cortisol will usually need to be administrated intravenously. During flu, the patient needs to take more salt and fluids. If diarrhea or vomiting is persistent he should be treated in an emergency room with intravenous saline and hydrocortisone. Patients with Addison’s disease needs regular follow up. Treating an Addison’s crisis is particularly challenging; it requires a quick response, which should include intravenous infusions of saline, sugar, and hydrocortisone. Pathogenesis: Addison’s disease occurs when more then 90% of adrenal gland tissue is destroyed (primary Addison’s disease). The destruction can occur due to various causes:
The idiopathic Addison’s disease is an autoimmune disease. There are the following pieces of evidence: Circumstantial evidence:
Indirect evidence: Murine model: Experimental autoimmune adrenalitis was produced in mice by immunizing 8 times in intervals of 30 days with syngeneic adrenal extract mixed with Klebsiella O3 lipopolysaccharide (KO3 LPS) as a potent adjuvant. Adrenalitis could be transfer by spleen cells from immunized mice to naïve donor. |
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Links to other Resources: www.adshg.org.uk www.addisons.org.au www.medhelp.org/nadf |
