Primary Aldosteronism Differential Diagnoses

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

Diagnostic Considerations

Consider the diagnosis of primary aldosteronism in all persons with hypertension (HTN) and hypokalemia. Making the correct diagnosis may be the only way to achieve adequate blood pressure control and thus, to prevent the sequelae of poorly controlled HTN.

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

Differential Diagnoses