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Consequences of ADPKD

Over time, enlarging cysts contribute to an increase in total kidney volume (TKV), damaging the kidney tissue and diminishing kidney function1-3

When autosomal dominant polycystic kidney disease (ADPKD) advances, the kidney progressively loses its ability to concentrate urine and function effectively.4

Loss of kidney function is irreversible5

The enlarging cysts cause compression and loss of the surrounding functional renal tissue, which can contribute to the progressive loss of kidney function, eventually leading to end-stage renal disease (ESRD).1,6

Cysts also damage the kidney by obstructing urine flow and causing intracellular inflammation and fibrosis, leading to progressive nephron loss.5

ADPKD is a systemic disease with renal and extra-renal manifestations.7–10

The most common extra-renal manifestation is hepatic cysts, and the most common causes of death in ADPKD patients are cardiac disease (36%) and infection (24%).7,11

  • ADPKD may lead to intracranial aneurysms. Ruptured intracranial aneurysms account for about 4% to 7% of all deaths in ADPKD patients12
  • ADPKD also takes an emotional toll. In a clinical study, 60% of ADPKD patients experienced depression13
  • As symptoms of ADPKD progress, they can affect a patient’s quality of life and place a burden on society14

Management of ADPKD

should focus on controlling associated symptoms of the illness1

ESRD and life expectancy

The development of renal insufficiency is highly variable in ADPKD.15

  • Renal failure has been reported in children, and yet other individuals with the disease may live a normal life expectancy without even knowing that they have ADPKD15
    • Cysts appearing at a younger age predicts a more rapid pace of kidney destruction and earlier onset of ESRD3
  • A 1984 report from Canada found that the probability of being alive with ADPKD and not having renal failure was 77% by age 50 years, 57% by age 58 years, and 52% by age 73 years15:
  1. Torres VE, Grantham JJ. Cystic diseases of the kidney. In: Taal MW, Chertow GM, Mardsen PA, Skorecki K, Yu ASL, Brenner BM, eds. Brenner & Rector’s The Kidney. Philadelphia, PA: Elsevier Saunders; 2012:1626-1667.

  2. Schrier RW. Randomized intervention studies in human polycystic kidney and liver disease. J Am Soc Nephrol. 2010;21(6):891-893. doi:10.1681/ASN.2010030262

  3. Braun WE. Autosomal dominant polycystic kidney disease: emerging concepts of pathogenesis and new treatments. Cleve Clin J Med. 2009;76(2):97-104.

  4. Meijer E, Boertien WE, Zietse R, Gansevoort RT. Potential deleterious effects of vasopressin in chronic kidney disease and particularly autosomal dominant polycystic kidney disease. Kidney Blood Press Res. 2011;34(4):235-244. doi:10.1159/000326902

  5. Grantham JJ, Mulamalla S, Swenson-Fields KI. Why kidneys fail in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2011;7(10):556-566.

  6. Harris PC, Torres VE. Polycystic kidney disease. Annu Rev Med. 2009;60:321-337.

  7. Halvorson CR, Bremmer MS, Jacobs SC. Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment. Int J Nephrol Renovasc Dis. 2010;3:69-83.

  8. Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369(9569):1287-1301.

  9. Torres VE, Harris PC. Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int. 2009;76(2):149–168. doi:10.1038/ki.2009.128 

  10. Li X, ed. Polycystic Kidney Disease. Brisbane, Australia. Codon Publications; 2015.

  11. Fick GM, Johnson AM, Hammond WS, Gabow PA. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995;5(12):2048-2056.

  12. Masoumi A, Reed-Gitomer B, Kelleher C, Bekheirnia MR, Schrier RW. Developments in the management of autosomal dominant polycystic kidney disease. Ther Clin Risk Manag. 2008;4(2):393-407.

  13. de Barros BP, Nishiura JL, Heilberg IP, Kirsztajn GM. Anxiety, depression, and quality of life in patients with familial glomerulonephritis or autosomal dominant polycystic kidney disease. J Bras Nefrol. 2011;33(2):120-128.

  14.  Blanchette CM, Matter S, Chawla A, Nellesen D, Rossetti S, Gutierrez B. Burden of autosomal dominant polycystic kidney disease: systematic literature review. Am J Pharm Benefits. 2015;7(2):e27-e36. https://www.ajpb.com/journals/ajpb/2015/ajpb_marchapril2015/burden-of-autosomal-dominant-polycystic-kidney-disease-systematic-literature-review.

  15. Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol. 2006;1(1):148-157.