supportive treatments are recommended with the aim of reducing morbidity and mortality associated with disease manifestations - therapies aim to slow the decline in renal volume to delay progression (1,2, 3)
besides lifestyle changes (low-salt diet, sufficient fluid intake, and no smoking), blood pressure control is the primary nonspecific treatment recommended by Kidney Disease -Improving Global Outcomes (KDIGO) for ADPKD patients
normalization of blood pressure, a salt-reduced diet, sufficient fluid intake (2-3 liters/day), avoidance of smoking and nephrotoxic agents such as nonsteroidal anti-inflammatory drugs, as well as restriction of caffeine were suggested (2)
early management of hypertension is important in reducing cardiovascular mortality, the incidence of left ventricular hypertrophy, mitral regurgitation, and to slow the progression of renal failure
tolvaptan (vasopressin V2 receptor antagonist) has demonstrated a slower decline than placebo in the eGFR over a one year period in patients with late-stage chronic kidney disease but is associated with elevations of bilirubin and alanine aminotransferase levels
evidence suggests patients who were treated with tolvaptan had a lower annual increase in total kidney volume, a slower rate of decline of kidney function, and prolonged life expectancy
Notes:
screening for a cerebral aneurysm is recommended at the time of ADPKD diagnosis in patients that are high risk (those with a family history of an aneurysm or intracranial hemorrhage in a first-degree relative)
Indications for screening in patients with good life expectancy include family history of ICA or subarachnoid hemorrhage, previous ICA rupture, high-risk professions (e.g., airline pilots) and patient anxiety despite adequate information (3)
Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF Jr, et al; Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP): Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2011; 6: 640-647.
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