Which conditions should be considered in the differential diagnosis of primary aldosteronism?

Updated: Mar 24, 2020
  • Author: Gabriel I Uwaifo, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
  • Print
Answer

Conditions to consider in the differential diagnosis of primary aldosteronism include the following:

  • HTN

  • Malignant HTN

  • Hypertensive encephalopathy

  • Hypokalemia

  • Metabolic alkalosis

  • Renal artery stenosis

  • Renovascular HTN

  • Low-renin essential HTN - Constitutes about 40% of essential HTN

  • Tobacco chewing

  • Carbenoxolone intoxication

  • Apparent mineralocorticoid excess (AME) syndrome

  • Various causes of secondary aldosteronism - Unlike primary aldosteronism, these causes are associated with elevated renin (plasma renin activity) levels

  • Chrétien syndrome - This rare syndrome is characterized by mineralocorticoid excess and adrenocortical HTN secondary to a pituitary adenoma producing pro-opiomelanocortin (POMC) [20]

  • Deoxycorticosterone (DOC)–secreting adrenal tumors

  • Renovascular ischemia

  • Preeclampsia (toxemia of pregnancy)

  • Renin-secreting tumor - These are rare tumors arising from the juxtaglomerular apparatus

  • Excessive licorice intake - In this situation, the glycyrrhizinic acid component inhibits 11beta-hydroxysteroid dehydrogenase, impairing conversion of cortisol to cortisone in the kidneys; hence, cortisol binds to mineralocorticoid receptors and acts as a mineralocorticoid

Additional select genetic/familial disorders and syndromes to consider include the following:

  • Gitelman syndrome - This is due to a defective sodium/chloride cotransporter (NCCT); it is basically a salt-losing tubulopathy with secondary aldosteronism

  • Barrter syndrome - This is a phenocopy of at least 3 distinct genetic defects (ie, hyperactivition of the sodium-potassium-dichloride cotransporter [NKCC2], the renal outer medullary potassium channel [ROMK1], or the renal epithelial chloride channel [ClCKb],the latter encoded by the barttin gene; this is also a salt-losing tubulopathy with secondary aldosteronism and is pathophysiologically similar to Gitelman syndrome

  • Gordon syndrome - This is due to inactivating mutations of the serine-threonine kinases WNK1 and WNK4 (“with no lysine [K]” kinases), leading to hypertension, hyperkalemia, mild hyperchloremia, acidosis, and suppressed plasma renin activity

  • Pseudoaldosteronism (Liddle syndrome) - This is a rare autosomal dominant disorder due to hyperactivating mutations of the renal epithelial sodium channel (ENaC), with excessive sodium reabsorption in the renal distal tubule; levels of renin and aldosterone are low

  • 11beta-hydroxysteroid dehydrogenase deficiency

  • Glucocorticoid resistance - This is due to inactivating mutations of the glucocorticoid receptor


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!