Acquired cystic disease of the kidney in adults
- Arlene B Chapman, MD
Arlene B Chapman, MD
- Professor of Medicine
- University of Chicago Pritzker School of Medicine
- Frederic F Rahbari-Oskoui, MD, MSCR
Frederic F Rahbari-Oskoui, MD, MSCR
- Associate Professor of Medicine
- Emory University School of Medicine
- William M Bennett, MD
William M Bennett, MD
- Medical Director
- Legacy Good Samaritan Transplant Services
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 . 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 .
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 . 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 . 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 .
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 .
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 . 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].
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