Primary adrenal insufficiency (PAI) is the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. It frequently manifests as Addison′s disease (AD) in adults and congenital adrenal hyperplasia (CAH) in children. 

Although recent years have witnessed diagnostic advances and the emergence of glucocorticoid and mineralocorticoid replacements, the adrenal crisis (AC) still remains a potentially lethal condition accounting for the increased mortality, not only in the first year of life but also throughout life. 

Occurrence of AD and an increased morbimortality rate of PAI can be observed if one fails to increase glucocorticoid doses during acute stress when there is a need for even greater amounts of glucocorticoids.

Brazil may show up to 1,500 deaths from AC in the coming decade if the mortality rate of 0.5 per 100 patient-years is considered, which means an alarming situation. The typical clinical features are described as hypotension and volume depletion. Other Nonspecific symptoms may also occur like fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain. 

The triggering factors are postulated to be gastrointestinal diseases, other infectious diseases, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. 

Suspected AC cases must be immediately managed with therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if required. It is preventable via patient education, precocious identification and adjusting the glucocorticoid dosage in stressor situations. Additionally, the emergency card, warning about acute glucocorticoid replacement, demonstrates a great value in decreasing the morbidity and mortality of AC.

Source: Arch Endocrinol Metab. 2021 Jul 16:2359-3997000000392. doi: 10.20945/2359-3997000000392. Epub ahead of print. PMID: 34283908.