Delayed diagnosis of primary aldosteronism in patients with autosomal dominant polycystic kidney diseases

Abstract

Hypertension is a frequent early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Several mechanisms can cause hypertension in ADPKD patients, although, primary aldosteronism (PA) as a possible manifestation of hypertension in ADPKD is extremely rare. We retrospectively reviewed the Taiwan Primary Aldosteronism Investigation (TAIPAI) database, which listed a total of 346 patients diagnosed with PA. Of these 346 patients, only three cases of concurrent PA and ADPKD were identified. These patients presented with hypertensive crisis and hypokalemia, and subsequent testing revealed aldosterone-producing adenomas (APAs) that were removed by laparoscopic adrenalectomy. Postoperatively, aldosterone-renin ratios (ARRs) and potassium levels normalized, and blood pressure improved. The diagnosis of PA in ADPKD is extremely challenging because multiple renal cysts can obscure the identification of adrenal adenomas, and ADPKD is associated with hypertension in almost all cases.(1) Because of frequent delays in the diagnosis of PA in ADPKD patients, future prospective studies to screen PA in hypertensive ADPKD patients may be necessary to evaluate the exact prevalence of coexistence of PA and ADPKD.

Keywords: Primary aldosteronism; autosomal dominant polycystic kidney disease; hypertensive crisis; hypokalemia.