Determining the etiology of primary adrenal insufficiency in adults

This algorithm reviews the approach to determining the etiology of primary adrenal insufficiency. It is based upon UpToDate content on diagnosis of adrenal insufficiency and causes of primary adrenal insufficiency.

21-OH-Abs: 21-hydroxylase antibodies; ALD: adrenoleukodystrophy; CT: computed tomography; PAI: primary adrenal insufficiency; VLCFA: very long-chain fatty acids.

* Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is not included in the algorithm, because it presents in infancy or childhood, not adulthood.

¶ The adrenocorticolytic drug mitotane causes PAI. Other drugs that inhibit cortisol biosynthesis (eg, ketoconazole, osilodrostat, metyrapone) do not usually cause clinically important PAI unless the patient has limited pituitary or adrenal reserve.

Δ In adults with autoimmune primary adrenal insufficiency, thyroid disease and type 1 diabetes are the most likely endocrine disorders to also be present (as part of polyglandular autoimmune syndrome type II).

◊ To review the characteristic adrenal CT findings of these disorders, refer to UpToDate topic on causes of primary adrenal insufficiency.

Adapted from: Bornstein SR, Allolio B, Arlt W, Barthel A, et al. Diagnosis and treatment of primary adrenal insufficiency: An Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 2016; 101:364

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Determining the etiology of primary adrenal insufficiency in adults