“Autosomal dominant polycystic kidney disease (ADPCKD) is the most common genetic kidney disease and accounts for 4.7% of end-stage kidney disease cases in America. Many patients with ADPCKD are asymptomatic, but early symptoms can include flank pain, gross hematuria, or recurrent urinary tract infections. The most common extrarenal manifestation of ADPCKD is hypertension, which can precipitate cardiovascular dysfunction, including left ventricular hypertrophy. Thus, early diagnosis and management of hypertension is crucial. The goal blood pressure should be <140/90 mm Hg in patients under the age of 60. All ADPCKD patients eventually develop a loss of renal function, and approximately 80% develop end-stage renal disease by age 70.
An ACE inhibitor like Lisinopril is the recommended first-line therapy. Angiotensin receptor blockers are acceptable in patients who cannot tolerate ACE inhibitors.” ABFM
Reference
N Engl J Med 2008;359(14):1477-1485.
Am Fam Physician 2014;90(5):303-307.