Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Acquired cystic disease of the kidney in adults

Arlene B Chapman, MD
Frederic F Rahbari-Oskoui, MD, MSCR
William M Bennett, MD
Section Editor
Ronald D Perrone, MD
Deputy Editor
Alice M Sheridan, MD


Chronic renal failure (particularly in patients on maintenance hemodialysis or peritoneal dialysis) is frequently associated with the development of multiple and bilateral renal cysts, which are usually <0.5 cm in diameter but can be as large as 2 to 3 cm [1-4]. The diagnosis of acquired cystic disease is established by ultrasonography, computed tomography (CT) scanning, or magnetic resonance imaging (MRI), although each procedure can have false-negative results. CT- and MR-based imaging offer increased sensitivity in detecting small cysts over ultrasonography [5]. A diagnosis requires involvement of both kidneys, with four or more cysts being present. Acquired cystic disease can be easily distinguished from autosomal dominant polycystic kidney disease (ADPKD) as the kidneys are small to normal in size, as opposed to the extremely large kidneys found in all ADPKD individuals with renal insufficiency.

Often, acquired cystic disease can be detected prior to end-stage renal disease (ESRD) and has been reported in patients with creatinine clearances as high as 70 mL/min. However, the majority of cases occur in patients with creatinine clearances <50 mL/min [6].

The incidence of acquired cystic disease increases progressively with duration of dialysis. In one study of 130 patients with advanced renal disease or ESRD, the incidence of multiple cysts was noted to be 7 percent in those with chronic renal failure and 22 percent in those on maintenance dialysis [3]. An additional 30 to 50 percent had one to three renal cysts, potentially representing an early form of the disease. The duration of dialysis was 15 months in patients with no cysts, 28 months in those with one to three cysts, and 49 months in those with acquired cystic disease [3]. Fifty to 80 percent of patients are affected after ≥10 years on dialysis [2,4]. Men and African Americans have been reported to be at much higher risk than women or Caucasians [1,7]. However, additional studies have estimated a 31 percent prevalence of acquired cystic disease in black African dialysis patients, similar to reports in other ethnic groups [8].

These observations suggest that the duration of chronic kidney disease (CKD) is a major risk factor for renal cyst development. In comparison, the etiology of the underlying renal disease does not appear to associate with the development of acquired cystic disease [1].


The pathogenesis of acquired cystic disease is incompletely understood. Nephron loss of any cause leads to compensatory hypertrophy in the remaining normal nephrons [1,2] (see "Secondary factors and progression of chronic kidney disease"). The cysts are limited to the kidney (in comparison with polycystic kidney disease [PKD]), suggesting that local, intrarenal events are of primary importance [1]. The cyst fluid, which is thought to derive from ultrafiltrate secreted into the cyst, typically has a composition similar to that in the plasma; this finding plus the presence of a brush border on the luminal membrane suggests that the cysts arise primarily from proliferation of proximal tubular epithelial cells [1,2].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Jul 01, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Grantham JJ. Acquired cystic kidney disease. Kidney Int 1991; 40:143.
  2. Ishikawa I. Acquired cystic disease: mechanisms and manifestations. Semin Nephrol 1991; 11:671.
  3. Narasimhan N, Golper TA, Wolfson M, et al. Clinical characteristics and diagnostic considerations in acquired renal cystic disease. Kidney Int 1986; 30:748.
  4. Matson MA, Cohen EP. Acquired cystic kidney disease: occurrence, prevalence, and renal cancers. Medicine (Baltimore) 1990; 69:217.
  5. Ferda J, Hora M, Hes O, et al. Computed tomography of renal cell carcinoma in patients with terminal renal impairment. Eur J Radiol 2007; 63:295.
  6. Liu JS, Ishikawa I, Horiguchi T. Incidence of acquired renal cysts in biopsy specimens. Nephron 2000; 84:142.
  7. Ishikawa I, Saito Y, Shikura N, et al. Ten-year prospective study on the development of renal cell carcinoma in dialysis patients. Am J Kidney Dis 1990; 16:452.
  8. Gnionsahe DA, Lagou DA, Tia WM. Prevalence of acquired cystic disease in black Africans on hemodialysis in West Africa. Saudi J Kidney Dis Transpl 2007; 18:114.
  9. Herrera GA. C-erb B-2 amplification in cystic renal disease. Kidney Int 1991; 40:509.
  10. Konda R, Sato H, Hatafuku F, et al. Expression of hepatocyte growth factor and its receptor C-met in acquired renal cystic disease associated with renal cell carcinoma. J Urol 2004; 171:2166.
  11. Oya M, Mikami S, Mizuno R, et al. C-jun activation in acquired cystic kidney disease and renal cell carcinoma. J Urol 2005; 174:726.
  12. Lien YH, Hunt KR, Siskind MS, Zukoski C. Association of cyclosporin A with acquired cystic kidney disease of the native kidneys in renal transplant recipients. Kidney Int 1993; 44:613.
  13. Truong LD, Krishnan B, Cao JT, et al. Renal neoplasm in acquired cystic kidney disease. Am J Kidney Dis 1995; 26:1.
  14. Levine E, Slusher SL, Grantham JJ, Wetzel LH. Natural history of acquired renal cystic disease in dialysis patients: a prospective longitudinal CT study. AJR Am J Roentgenol 1991; 156:501.
  15. Rainio J, De Giorgio F, Carbone A. Death from renal cyst: spontaneous or traumatic rupture? Am J Forensic Med Pathol 2006; 27:193.
  16. Moore AE, Kujubu DA. Spontaneous retroperitoneal hemorrhage due to acquired cystic kidney disease. Hemodial Int 2007; 11 Suppl 3:S38.
  17. Sarasin FP, Wong JB, Levey AS, Meyer KB. Screening for acquired cystic kidney disease: a decision analytic perspective. Kidney Int 1995; 48:207.
  18. Farivar-Mohseni H, Perlmutter AE, Wilson S, et al. Renal cell carcinoma and end stage renal disease. J Urol 2006; 175:2018.
  19. Denton MD, Magee CC, Ovuworie C, et al. Prevalence of renal cell carcinoma in patients with ESRD pre-transplantation: a pathologic analysis. Kidney Int 2002; 61:2201.
  20. Terasawa Y, Suzuki Y, Morita M, et al. Ultrasonic diagnosis of renal cell carcinoma in hemodialysis patients. J Urol 1994; 152:846.
  21. Takagi T, Kondo T, Izuka J, et al. Prognosis and characteristics of renal cell carcinoma in hemodialysis patients: bilateral occurrence does not influence cancer-specific survival. Int J Urol 2011; 18:806.
  22. Smith JW, Sallman AL, Williamson MR, Lott CG. Acquired renal cystic disease: two cases of associated adenocarcinoma and a renal ultrasound survey of a peritoneal dialysis population. Am J Kidney Dis 1987; 10:41.
  23. MacDougall ML, Welling LW, Wiegmann TB. Prediction of carcinoma in acquired cystic disease as a function of kidney weight. J Am Soc Nephrol 1990; 1:828.
  24. Chandhoke PS, Torrence RJ, Clayman RV, Rothstein M. Acquired cystic disease of the kidney: a management dilemma. J Urol 1992; 147:969.
  25. Kojima Y, Takahara S, Miyake O, et al. Renal cell carcinoma in dialysis patients: a single center experience. Int J Urol 2006; 13:1045.
  26. Marple JT, MacDougall M, Chonko AM. Renal cancer complicating acquired cystic kidney disease. J Am Soc Nephrol 1994; 4:1951.
  27. Hughson MD, Schmidt L, Zbar B, et al. Renal cell carcinoma of end-stage renal disease: a histopathologic and molecular genetic study. J Am Soc Nephrol 1996; 7:2461.
  28. Inoue H, Nonomura N, Kojima Y, et al. Somatic mutations of the von Hippel-Lindau disease gene in renal carcinomas occurring in patients with long-term dialysis. Nephrol Dial Transplant 2007; 22:2052.
  29. Schwarz A, Vatandaslar S, Merkel S, Haller H. Renal cell carcinoma in transplant recipients with acquired cystic kidney disease. Clin J Am Soc Nephrol 2007; 2:750.
  30. Taylor AJ, Cohen EP, Erickson SJ, et al. Renal imaging in long-term dialysis patients: a comparison of CT and sonography. AJR Am J Roentgenol 1989; 153:765.
  31. Takebayashi S, Hidai H, Chiba T, et al. Using helical CT to evaluate renal cell carcinoma in patients undergoing hemodialysis: value of early enhanced images. AJR Am J Roentgenol 1999; 172:429.
  32. Mühlfeld AS, Lange C, Kroll G, et al. Pilot study of non-contrast-enhanced MRI vs. ultrasound in renal transplant recipients with acquired cystic kidney disease: a prospective intra-individual comparison. Clin Transplant 2013; 27:E694.
  33. Paudice N, Zanazzi M, Agostini S, et al. Contrast-enhanced ultrasound assessment of complex cystic lesions in renal transplant recipients with acquired cystic kidney disease: preliminary experience. Transplant Proc 2012; 44:1928.
  34. Chung WY, Nast CC, Ettenger RB, et al. Acquired cystic disease in chronically rejected renal transplants. J Am Soc Nephrol 1992; 2:1298.
  35. Rudge CJ. Acquired cystic disease of the kidney: serious or irrelevant? Br Med J (Clin Res Ed) 1986; 293:1186.
  36. Fick GM, Gabow PA. Hereditary and acquired cystic disease of the kidney. Kidney Int 1994; 46:951.
  37. Ishikawa I, Honda R, Yamada Y, Kakuma T. Renal cell carcinoma detected by screening shows better patient survival than that detected following symptoms in dialysis patients. Ther Apher Dial 2004; 8:468.