What is the pathophysiology of adrenocortical insufficiency in adrenal crisis?

Updated: Feb 14, 2018
  • Author: Lisa Kirkland, MD, FACP, FCCM, MSHA; Chief Editor: George T Griffing, MD  more...
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Answer

Primary adrenocortical insufficiency occurs when the adrenal glands fail to release adequate amounts of these hormones to meet physiologic needs, despite release of ACTH from the pituitary. Infiltrative or autoimmune disorders are the most common cause, but adrenal exhaustion from severe chronic illness also may occur.

Secondary adrenocortical insufficiency occurs when exogenous steroids have suppressed the hypothalamic-pituitary-adrenal (HPA) axis. Too rapid withdrawal of exogenous steroid may precipitate adrenal crisis, or sudden stress may induce cortisol requirements in excess of the adrenal glands' ability to respond immediately. In acute illness, a normal cortisol level may actually reflect adrenal insufficiency because the cortisol level should be quite elevated.

Bilateral massive adrenal hemorrhage (BMAH) occurs under severe physiologic stress (eg, myocardial infarction, septic shock, complicated pregnancy) or with concomitant coagulopathy or thromboembolic disorders.

Hahner et al investigated the frequency and causes of, as well as the risk factors for, adrenal crisis in patients with chronic adrenal insufficiency. Using a disease-specific questionnaire, the authors analyzed data from 444 patients, including 254 with primary adrenal insufficiency and 190 with secondary adrenal insufficiency. At least one adrenal crisis was reported by 42% of patients, including 47% of those with primary adrenal insufficiency and 35% of patients with the secondary condition. GI infection and fever were the most common precipitating causes of crisis. Identified risk factors for adrenal crisis were, for patients with primary adrenal insufficiency, concomitant nonendocrine disease, and for patients with secondary adrenal insufficiency, female sex and diabetes insipidus. [3]

A study from the Netherlands, by Smans et al, of patients with adrenal insufficiency, found the existence of comorbidity to be the most important risk factor for adrenal crisis, with infections being the most common precipitating factors. [4]

A Japanese study, by Iwasaku et al, found that of 504 patients with adrenal crisis, only about one third had preexisting primary or central adrenal insufficiency. In the rest of the patients, comorbidities included cardiovascular disease (31.6%), infection (18.8%), diabetes (16.5%), cancer (19.1%), hypothyroidism (7.0%), autoimmune disease (9.3%), peptic ulcer (6.1%), chronic obstructive pulmonary disease or asthma (6.1%), and renal failure (6.1%). [5]


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