Adrenocortical hyperplasia: A multifaceted disease

Best Pract Res Clin Endocrinol Metab. 2020 May;34(3):101386. doi: 10.1016/j.beem.2020.101386. Epub 2020 Feb 4.


Adrenocortical hyperplasia may develop in different contexts. Primary adrenal hyperplasia may be secondary to primary bilateral macronodular adrenocortical hyperplasia (PBMAH) or micronodular bilateral adrenal hyperplasia (MiBAH) which may be divided in primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). Both lead to oversecretion of cortisol and potentially to Cushing's syndrome. Moreover, adrenocortical hyperplasia may be secondary to longstanding ACTH stimulation in ACTH oversecretion as in Cushing's disease, ectopic ACTH secretion or glucocorticoid resistance syndrome and congenital adrenal hyperplasia secondary to various enzymatic defects within the cortex. Finally, idiopathic bilateral adrenal hyperplasia is the most common cause of primary aldosteronism. We will discuss recent findings on the multifaceted forms of adrenocortical hyperplasia.

Keywords: Cushing's syndrome; adrenocortical hyperplasia; congenital adrenal hyperplasia; micronodular adrenal hyperplasia; primary bilateral macronodular adrenal hyperplasia; primary pigmented nodular adrenal disease.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Diseases* / classification
  • Adrenal Cortex Diseases* / diagnosis
  • Adrenal Cortex Diseases* / etiology
  • Adrenal Gland Neoplasms / complications
  • Adrenal Gland Neoplasms / diagnosis
  • Adrenal Glands / pathology*
  • Adrenal Hyperplasia, Congenital / diagnosis
  • Adrenal Hyperplasia, Congenital / etiology
  • Cushing Syndrome / diagnosis
  • Cushing Syndrome / etiology
  • Humans
  • Hydrocortisone / metabolism
  • Hyperaldosteronism / complications
  • Hyperaldosteronism / diagnosis
  • Hyperplasia / diagnosis
  • Hyperplasia / etiology
  • Metabolism, Inborn Errors / complications
  • Metabolism, Inborn Errors / diagnosis
  • Receptors, Glucocorticoid / deficiency


  • Receptors, Glucocorticoid
  • Hydrocortisone

Supplementary concepts

  • Glucocorticoid Receptor Deficiency