Addison's Disease

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Original Editors -  Elizabeth Bohnert from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Addison’s disease is another name for primary chronic adrenal insufficiency.  It is a condition where the adrenal cortex (the outer layer of the adrenal gland that produces mineralocorticoids, glucocorticoids, and androgens) is progressively destroyed, resulting in decreased secretions of the hormones.  Cortisol, a glucocorticoid, and aldosterone, a mineralcorticoid, are the primary hormones that are decreased with this disease, causing body wide metabolic disorders and fluid imbalances.

[Image courtesy of Britannica.com. Available at http://www.britannica.com/EBchecked/topic/6405/adrenal-gland]

Prevalence

Addison’s disease occurs in about 4 out of 100,000 Americans each year.  Even though it is seen throughout the lifespan in both genders, it most commonly occurs in middle-aged white females.

Characteristics/Clinical Presentation

Addison’s disease will develop insidiously, with the signs of the disease not developing “until at least 90% of both glands is destroyed and the levels of circulating glucocorticoids and mineralocorticoids are significantly decreased.”  Some of the clinical signs and symptoms of Addison’s disease include:

• Darkened pigmentation of the skin (especially of the mouth and scars), due to increased secretion of melanin-secreting hormone (MSH) corresponding with increased secretion of ACTH, which is released to try to stimulate the work of the adrenal glands   

File:Hyperpigmentation.jpg

Hyperpigmentation of buccal mucosa in Addison's disease

• Slowly developing weakness and fatigue

• Hypotension due to increased sodium excretion from decreased aldosterone secretion

• Severe abdominal, low back, or leg pain

• Gastrointestinal disturbances such as nausea, vomiting, anorexia, weight loss, and diarrhea

• Hypoglycemia due to decreased glucocorticoids causing decreased gluconeogenesis

• Decreased stress tolerance (infections, trauma, surgery, etc.)

• Salt craving

[Image courtesy of University of South Carolina School of Medicine. Available at http://pathmicro.med.sc.edu/ghaffar/tolerance2000.htm]

Associated Co-morbidities

Addison's disease can occur alone, or it can occur along with other autoimmune disorders, leading to polyendocrine syndrome.

• Type I Diabetes Mellitus
• Hypoparathyroidism
• Thyroiditis
• Pernicious Anemia
• Vitiligo (a loss of pigment on areas of the skin)
• Chronic Active Hepatitis

Osteoporosis is more common among people with Addison's disease compared to healthy individuals because of the medical management with long-term steroid replacement therapy.  Steroids have several effects on bones including increasing osteoclastic activity, decreasing osteoblastic formation, and decreasing the absorption of calcium in the intestines.

Bone Mineral Density T-Scores

Status	T-Scores
Normal	-1.0 or above
Osteopenia	-1.0 to -2.5
Osteoporosis	-2.5 or less
Severe Osteoporosis	-2.5 or less with 1 or more fragility fractures

Medications

Acute Therapy:

• Intravenously administered hydrocortisone (100 mg), repeated every 6 hours for the first 24 hours

• Intravenously administered rapid infusion of saline (2-4L) during the first 12 hours

Maintenance Therapy:

• Glucocorticoid Replacement: 15-20 mg of hydrocortisone daily, divided into 3 or 4 doses to simulate normal adrenal secretions

• Mineralocorticoid Replacement: 0.05-0.1 mg of fludrocortisones daily

Diagnostic Tests/Lab Tests/Lab Values

Test Results That Suggest Addison's Disease

Test	Result
Blood Chemistry
Serum Na	< 135 mEq/L
Serum K	> 5 mEq/L
Ratio of Serum Na:K	<30:1
Plasma Glucose, fasting	< 50 mg/dL
Plasma HCO3	< 15-20 mEq/L
BUN	> 20 mg/dL
Hematology
Hct	Elevated
WBC Count	Low
Lymphocytes	Relative lymphocytes
Eosinophils	Increased
Imaging
	Evidence of calcification in adrenal areas Renal TB Pulmonary TB

[Chart courtesy of Merck.com.  Available at http://merck.com/mmpe/sec12/ch153/ch153b.html]

Causes

There are a number of causes for Addison’s disease, however “90% of all cases are attributable to one of four diseases: autoimmune adrenalitis, tuberculosis, the acquired immune deficiency syndrome (AIDS), or metastatic cancers.”  The primary sites of the metastases to the adrenal glands usually arise from carcinomas of the lungs or breasts.

Systemic Involvement

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Medical Management (current best evidence)

Refer to Medications

Physical Therapy Management (current best evidence)

Unfortunately, there is very little information and research pertaining to the physical therapy management of patients with Addison's disease.  The APTA's Hooked on Evidence yielded not a single article with the term "Addison's disease." Then EBSCOhost, GoogleScholar, and ProQuest were all used with the search terms "Addison's disease" and "physical therapy" or "exercise" and there was only one pertinent journal article.  Jakobi et al found that women with Addison's disease had similar maximum voluntary force of the quadriceps muscle compared to the control group of health individuals, however they had "altered contractile properties and decreased endurance compared with controls." Obviously, more research needs to be performed to determine the proper interventions that physical therapists should utilize to treat patient's with Addison's disease and also determine any limitations to treating these patients.

Luckily, Goodman and Fuller offer some key points for the physical therapy management of patients with Addison's disease:

• Since these patients have a decreased response to stress, physical stress should be minimized as much as possible during activity and the treatment program should be "very gradually progressed" as the individual feels comfortable
• Aquatics therapy should NOT be utilized with these patients because the heat and humidity of the pool will place increased demands of cortisol on their bodies to increase their blood pressure
• Vital signs should be constantly monitored, especially when initiating or progressing an exercise program
• Monitor for the signs of an impending addisonian crisis, which includes "dizziness, nausea, profuse sweating, elevated heart rate, and tremors or shaking"
• Patients should be referred back to the physician if there is a suspected infection, since an infection places extra stress on the patient

Alternative/Holistic Management (current best evidence)

• A combination of Barago officinalis (Borage), Eleutherococcus senticosis (Siberian Ginseng), and Astragalus membranaceous (Huang Qi) help support the function of the adrenal glands and assist the body in dealing with normal stressors

• Ginger works as an anti-nausea medication and helps to decrease physicial and emotional stress

• Liquorice improves the activity of mineralocorticoids

Differential Diagnosis

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Case Reports

Addison’s disease presenting with idiopathic intracranial hypertension in 24-year-old woman: a case report [view article at Journal of Medical Case Reports]

Premature Mortality in Patients with Addison’s Disease: A Population-Based Study  [view article at The Journal of Clinical Endocrinology & Metabolism]

A three-year-old boy with X-linked adrenoleukodystrophy and congenital pulmonary adenomatoid malformation: a case report [view article at Journal of Medical Case Reports]

Resources

National Adrenal Diseases Foundation: www.nadf.us

Mayo Clinic: www.mayoclinic.com

The Canadian Addison Society: www.addisonsociety.ca

National Endocrine and Metabolic Diseases Information Service: www.endocrine.niddk.nih.gov

Recent Related Research (from Pubmed)

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