Management of ADPKD

• 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 (4,5)
  • normalization of blood pressure, a salt-reduced diet, sufficient fluid intake (2-3 litres/day), avoidance of smoking and nephrotoxic agents such as nonsteroidal anti-inflammatory drugs, as well as restriction of caffeine were suggested (6)
    • 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 (3)

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 haemorrhage in a first-degree relative) but widespread pre-symptomatic screening for intracranial aneurysms is not justified (4)
  • Indications for screening in patients with good life expectancy include family history of ICA or subarachnoid haemorrhage, previous ICA rupture, high-risk professions (e.g., airline pilots) and patient anxiety despite adequate information (4)

Reference:

1. Gimpel C, Bergmann C, Bockenhauer D, et al. International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol. 2019 Nov;15(11):713-726.
2. Torra R. Recent advances in the clinical management of autosomal dominant polycystic kidney disease. F1000Res. 2019 Jan 29;8
3. 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.
4. Chapman AB, Devuyst O, Eckardt KU, 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.
5. Ong AC, Devuyst O, Knebelmann B, et al. Autosomal dominant polycystic kidney disease: the changing face of clinical management. Lancet. 2015 May 16;385(9981):1993-2002.
6. Belibi FA, Wallace DP, Yamaguchi T, et al. The effect of caffeine on renal epithelial cells from patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2002 Nov;13(11):2723-9.