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Acquired Cystic Kidney Disease

Definition

Acquired cystic kidney disease (ACKD) is characterized by the development of multiple bilateral cysts in the kidneys of patients with chronic kidney disease without a history of a hereditary cystic disease (Choyke et al., 2000).

Epidemiology:

On average, 34% with end-stage renal disease present with ARCD, depending on the time on dialysis: 10% (under one year) up to 80% (over ten years).

Etiology of Acquired Cystic Kidney Disease

Tubular obstruction caused by fibrosis, oxalate crystals, or vascular occlusions leads to renal cysts. Secondly, cystogenic and/or carcinogenic substances of uremia (e.g., lack of elimination of growth factors) are responsible. The development of renal cysts correlates with the duration of end-stage renal disease; renal transplantation improves ACKD. After graft failure, the activity of ACKD increases again and may also affect the kidney transplant. There is a relevant risk of malignancy; up to 1% develop a renal cell carcinoma, and the relative risk is 5–50 times increased.

Pathology

Gross pathology:

Bilateral cortical cysts, on average 0.5 to 1 cm in size. Calcium oxalate crystals are often found in the cysts, which caused the nephrons to clog and induced the cysts.

Risk of malignancy:

Renal adenomas (1–3 cm of size) often develop from the renal cysts. If a renal mass develops a size of >3 cm, renal cell carcinoma is likely.

Signs and Symptoms

Flank pain, cyst infections, hematuria, risk of malignancy.

Diagnosis of Acquired Cystic Kidney Disease

Renal Ultrasound Imaging:

Renal ultrasound imaging shows small kidneys with multiple cysts. Signs of a cyst infection are a thickened cyst wall and hyperechogenic cysts. Renal tumors may be visible.

Abdominal CT Scan or MRI:

Indicate CT scan or MRI imaging if suspicious cysts are seen in ultrasound imaging. Prefer MRI imaging in patients with chronic kidney disease without dialysis.

Differential Diagnosis

Autosomal dominant polycystic kidney disease (ADPKD): the kidneys have usually a volume >300 ml.

Therapy of Acquired Cystic Kidney Disease

• Infected cyst: percutaneous drainage with pigtail catheter or nephrectomy.
• Bleeding or hematuria: consider peritoneal dialysis to avoid anticoagulation, embolisation of bleeding vessels, nephrectomy.
• Renal tumors: partial nephrectomy for patients with relevant renal function, otherwise radical nephrectomy.

References

Choyke 2000 CHOYKE, P. L.: Acquired cystic kidney disease.
In: Eur Radiol
10 (2000), Nr. 11, S. 1716–21

  German version: Erworbene zystische Nierenerkrankung