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Addison's Disease (Primary Adrenal Insufficiency) - Coggle Diagram
Addison's Disease
(Primary Adrenal Insufficiency)
Pathogenesis
Caused by inability of adrenal cortex to produce hormones
Autoimmune destruction of adrenal cortex can be triggered by a random environmental factor in those genetically susceptible
Chronic infection (such as tuberculosis, sepsis, HIV) is the major cause of Addison's in underdeveloped countries
Less common causes include:
spread of cancer to adrenal glands
bleeding into adrenal glands
In developed countries, the most common cause of Addison's is autoimmune destruction of adrenal glands
Starts with decreased cortisol production then decreased aldosterone, which eventually cause increase of ACTH and renin because there's a loss of negative feedback mechanism
Incidence/Prevalence
Occurrence of Addison's disease is rare
Approximately 1 in 100,000 people in U.S. have Addison's disease
Estimated to be 40-60 people per 1 million of general population
Many cases go undiagnosed because nonspecific symptoms make it hard to identify
Occurs in both sexes and all age groups - occurs most often in individuals between 30-50 years old
Risk Factors
Having other autoimmune disorders
Grave's disease
Type 1 diabetes mellitus
Cancer
Anticoagulants (blood thinners)
Chronic Infections
Surgery removing part of adrenal gland
Diagnostics
Blood tests - measure blood level of sodium, potassium, cortisol, adrenocorticotropic hormone, and anti-adrenal antibodies
ACTH stimulation test - measures level in cortisol in blood before and after injection of synthetic ACTH
Insulin-induced hypoglycemia test - checks blood glucose and cortisol levels
Imaging tests - CT scan of abdomen to check size of adrenal glands or to find abnormalities
Clinical Manifestations
Extreme fatigue
Low blood pressure/fainting
Hyponatremia/salt craving
Hypoglycemia (low blood sugar)
Nausea, diarrhea, vomiting
Muscle/joint pain
Irritability
Depression or behavioral symptoms
Adult women
body hair loss
sexual dysfunction
Weight loss/decreased appetite
Hyperpigmentation (skin darkening from ACTH)
Gradual onset of several nonspecific symptoms:
Treatment
All treatment involves medication - specifically hormone therapy to compensate for insufficient hormone production from the adrenal cortex
Hydrocortisone (Cortef), prednisone or methylprednisolone - replaces cortisol
Fludrocortisone acetate - replaces aldosterone
Appropriate salt intake - especially when exercising, in hot weather, with GI upsets (diarrhea)
References
Munir S, Waseem M. Addison Disease. [Updated 2020 Jul 5]. In: StatPears [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan.
https://www.ncbi.nlm.nih.gov/books/NBK441994
Griffing, G. (2020, May 28).
Addison Disease Clinical Presentation: History, Physical, Causes
.
https://emedicine.medscape.com/article/116467-clinical
Husebye, E.,& Lovas, K. (2009). Pathogenesis of primary adrenal insufficiency.
Best practice & research. Cinical endocrinology & metabolism, 23(2), 147-157.
https://doi.org/10.1016/j.beem.2008.09.004