2008 Update

Laboratory Endocrine Testing Guidelines:

ADRENAL INSUFFICIENCY (ADDISONS DISEASE)
GUIDELINE GOALS
To assist the practitioner in the initial laboratory investigation in patients with suspected Addisons disease To optimize the use of diagnostic laboratory tests

Administered by the Alberta Medical Association

BACKGROUND
The commonest cause of adrenal insufficiency is Addisons disease, a rare endocrine disorder that affects about 1 in 100,000 people. Addisons disease occurs in all age groups and afflicts men and women equally. Disease occurs more frequently in families predisposed to autoimmune endocrinopathies, e.g., thyroid, Type 1 Diabetes Mellitus. Additionally, a normal response in the short ACTH stimulation tests does not exclude secondary (pituitary) insufficiency as a cause. Addisonian crisis is a catastrophic complication of adrenocortical insufficiency, which can develop rapidly. The patient usually develops adrenocorticol insufficiency symptoms followed by profound hypotension. The patient may remain alert. Because this crisis is life threatening, urgent treatment and consultation cannot await laboratory results.1-4

RECOMMENDATIONS
Addisons Disease Clinical Features

Loss of appetite and weight loss Chronic worsening fatigue and muscle weakness Low blood pressure which falls further when

standing, causing dizziness or fainting Darkening of the skin in exposed and non-exposed parts of the body, particularly on skin creases and scars Nausea, vomiting, and diarrhea occur in about 50% of cases Hypoglycemia (more severe in children than in adults)

Causes: Primary Adrenal Insufficiency Seventy percent of reported cases are due to autoimmune disorders Tuberculosis accounts for about 20% of cases in developed countries Less common causes of primary adrenal URGENT CONSULTATION IS RECOMMENDED. insufficiency are chronic infections (mainly As soon as the stimulation test is completed, gluAIDS and fungal infections), hemorrhage cocorticoids can be administered as a life saving (secondary to anticoagulant therapy), cancer, measure amyloidosis, and adrenalectomy Synthetic ACTH stimulation testing is required for diagnosis Secondary Adrenal Insufficiency Low random serum cortisol levels have a poor pre- Lack of pituitary adrenocorticotropin (ACTH) dictive value and are not recommended. A serum cortisol with or without ACTH stimulation which REFERENCES exceeds 500 - 550 nmol/L excludes adrenal insufficiency1 1. Oeklers W. Adrenal insufficiency. New England Journal of Medicine, 1996; 335: 1206-1212. A normal response in the short ACTH stimulation 2. Grinspoon SK, Biller BMK. Laboratory test does not exclude secondary (pituitary) insufassessment of adrenal insufficiency. Journal of ficiency as a cause Clinical Endocrinology and Metabolism, 1994; 79: 923-931. 3. Chodosh LA, Dnaiels GH. Addisons disease. PRACTICE POINT Endocrinologist, 1993; 3: 166-181. 4. Snow K, Jiang NB, Kao FC, Scherthauer BW. If the diagnosis of Addisons Disease is Biochemical evaluation of adrenal dysfunction: strongly suspected, treatment should be the laboratory perspective. Mayo Clinic Proc, instituted immediately. 1992; 67: 1055-1065.

Toward Optimized Practice (TOP) Program

Arising out of the 2003 Master Agreement, TOP succeeds the former Alberta Clinical Practice Guidelines program, and maintains and distributes Alberta CPGs. TOP is a health quality improvement initiative that fits within the broader health system focus on quality and complements other strategies such as Primary Care Initiative and the Physician Office System Program. The TOP program supports physician practices, and the teams they work with, by fostering the use of evidence-based best practices and quality initiatives in medical care in Alberta. The program offers a variety of tools and out-reach services to help physicians and their colleagues meet the challenge of keeping practices current in an environment of continually emerging evidence. TO PROVIDE FEEDBACK The Endocrine Working Group is a multidisciplinary team composed of family physicians, internal medicine specialists, laboratory physicians and laboratory technologists. The Working Group encourages your feedback. If you need further information or if you have difficulty applying this guideline, please contact: Toward Optimized Practice Program 12230 - 106 Avenue NW EDMONTON, AB T5N 3Z1 T 780. 482.0319 TF 1-866.505.3302 F 780.482.5445 E-mail: cpg@topalbertadoctors.org Endocrine Guidelines, April 1998 Reviewed and revised January 2008