management of ADPKD

Last edited 07/2018 and last reviewed 07/2018

  • 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)

Reference: