Incidence of adrenal crisis in patients with adrenal insufficiency

Abstract

AC still occurs relatively frequent in patients with AI, mostly precipitated by infections and particularly in patients with high comorbidity. This should be taken into account in the education and follow-up of patients with AI.

MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

“…A life-threatening adrenal crisis is, unfortunately, a common event in PAI on replacement therapy. Recent studies provide evidence of an annual incidence of adrenal crisis of 5-17% (16,99,100,101,102,103) and show that 47% of PAI patients have experienced at least one acute adrenal crisis after diagnosis (100). In the first prospective study on adrenal crisis a total of 423 patients (PAI, n = 221; secondary adrenal insufficiency (SAI), n = 202) were followed up for 2 years; 10 patients died during follow-up (PAI, n = 5; SAI, n = 5), and at least 4 deaths were associated with adrenal crisis (0.5 adrenal-crisis-related deaths per 100 patients-years) (101).…”

“…Other listed causes are surgery, trauma, emotional stress and cessation of glucocorticoid substitution. Concomitant diseases (99,100,102) and previous adrenal crisis (101) increase the frequency. Even a minor stomach upset can provoke an Addisonian crisis, and in the event of an emergency medical staff must react with prompt administration of intravenous glucocorticoids and saline infusions.…”

Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan

“…In spite of preventive efforts, rapid deterioration of disease course or inadequate management results in the development of adrenal crisis [5]. The incidence of adrenal crisis has been reported to be between 5.2 and 8.3 per 100 patient-years (PYs) in patients with adrenal insufficiency of various ages, countries, and etiologies [5-9]. For children, Odenwald et al [6] conducted a prospective follow-up study of 102 Bavarian children aged 0–6 years with 21-OHD and estimated the incidence of adrenal crisis at 6.5 per 100 PYs (95% confidence interval [CI] 4.6–8.8).…”

Conduct protocol in emergency: Acute adrenal insufficiency

“…Other causes are displayed in Chart 1. 3,7,8,11,[16][17][18] The physician should always be alert to any changes in the vital signs of a patient with known chronic AI. Being aware and knowing how to correlate certain signs and symptoms that suggest acute AI is also necessary, as over 25% of patients with Addison's disease are diagnosed at the time of an adrenal crisis, probably triggered by a stressful event (surgery, infection or trauma).…”

“…Being aware and knowing how to correlate certain signs and symptoms that suggest acute AI is also necessary, as over 25% of patients with Addison's disease are diagnosed at the time of an adrenal crisis, probably triggered by a stressful event (surgery, infection or trauma). 4,16,18 Adrenal crisis should be included in the differential diagnosis when a patient enters the emergency room with hypotension that does not respond to the administration of volume or vasopressors, worsening on standing position, sometimes leading to shock, associated with nonspecific symptoms such as anorexia, nausea, vomiting, pain unexplained abdominal bloating, constipation, fever secondary to an infectious process, apathy, confusion, and drowsiness, which may progress to coma. 3,9,13,14,17,18,20 A suggestive sign of past Addison's disease, which can help make the diagnosis, is hyperpigmentation of mucous membranes and skin by melanin due to the excessive production of alpha-MSH (melanocortin stimulating hormone) derived from the same precursor protein of ACTH called POMC (pro-opiomelanocortin).…”