management of ADPKD

Last edited 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:

• Barnawi RA et al. Is the light at the end of the tunnel nigh? A review of ADPKD focusing on the burden of disease and tolvaptan as a new treatment. Int J Nephrol Renovasc Dis. 2018; 11: 53-67.
• 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.
• Chapman AB et al. Autosomal Dominant Polycystic Kidney Disease (ADPKD): Executive Summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2015 Jul; 88(1): 17-27.