7911 - Addison's disease (adrenocortical insufficiency)

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Alternate Names: Adrenal insufficiency, adrenal failure

Definition

Addison's disease results from a decrease in function of the cortex of the adrenal gland, and the loss of its principal hormone, cortisol.

Etiology

There are primary and secondary types of the condition:

  • Primary type makes up 70% of the cases. It occurs secondary to a wasting of the cortex by an autoimmune process, surgical removal of the adrenal gland, infectious diseases, e.g., tuberculosis, tumors, and hemorrhage.

  • Secondary type occurs secondary to or from steroids generated within the body via a tumor, or use of steroids over a long period of time resulting in hypopituitarism.

Signs & Symptoms

Early features include: weight loss, hypotension, anorexia, progressive weakness, and fatigue. In primary forms, skin pigmentation results in a bronze appearance, and black freckles appear over the head and neck. However, the bronze pigmentation may not be present in primary forms that occur rapidly. Dehydration, hyponatremia, and hyperkalemia may lead to profound shock.

Tests

Initial tests may show hyponatremia and hyperkalemia. Upon the administration of an adrenocorticotropic hormone (ACTH) stimulation test, failure of the plasma cortisol to increase and failure of the urine to excrete cortisol demonstrates adrenal insufficiency. A positive response to ACTH demonstrates a functioning adrenal gland and indicates probable pituitary disease.

Treatment

All patients with decreased adrenal function must have corticosteroid replacement with cortisone or hydrocortisone for life. Some patients may require mineralocorticoids. A delay in starting therapy could lead to death.

Residuals

Patients who receive proper treatment have an excellent prognosis. Those that require mineralocorticoids may, as a result of their use, experience hypokalemia, cardiac enlargement, and even congestive heart failure (CHF).

Special Considerations

  • Consider service connection on a presumptive basis as a chronic condition (38 CFR §3.307§3.309 (a)).

Notes

  • An Addisonian “crisis” consists of the rapid onset of peripheral vascular collapse (with acute hypotension and shock), with findings that may include: anorexia; nausea; vomiting; dehydration; profound weakness; pain in abdomen, legs, and back; fever; apathy, and depressed mentation with possible progression to coma, renal shutdown, and death.

  • An Addisonian “episode,” for VA purposes, is a less acute and less severe event than an Addisonian crisis and may consist of anorexia, nausea, vomiting, diarrhea, dehydration, weakness, malaise, orthostatic hypotension, or hypoglycemia, but no peripheral vascular collapse.

  • Tuberculous Addison's disease will be evaluated as active or inactive tuberculosis. If inactive, these evaluations are not to be combined with the graduated ratings of 50 percent or 30 percent for non-pulmonary tuberculosis specified under § 4.88b. Assign the higher rating.