Acquired Cystic Kidney Disease Clinical Presentation

Updated: Mar 13, 2020
  • Author: Manish Suneja, MD, FASN, FACP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Presentation

History

Acquired cystic kidney disease has been described in nearly every type of kidney disease that causes progressive renal insufficiency, with the exception of hereditary cystic disorders. The incidence and the number and size of cysts correlate with the number of years the patient is on dialysis.

In the early stages, acquired cystic kidney disease does not produce symptoms; it is usually discovered incidentally in the course of abdominal imaging procedures. Patients with more advanced disease may present with signs and symptoms due to cyst hemorrhage or infection, or distant metastasis from a malignant renal neoplasm.

Hemorrhagic cysts can occur in up to 50% of patients with acquired cystic kidney disease, and may present as flank pain and gross hematuria. [10] Rarely, patients may also develop spontaneous retroperitoneal hemorrhage, resulting in hemorrhagic shock, which may be fatal. [14, 15]

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Physical

Acquired cystic kidney disease is usually bilateral. The kidneys are rarely palpable but may become palpable after a bleed. See the table below.

Table 1. Comparison of Findings in Acquired Cystic Kidney Disease Versus Autosomal Dominant Polycystic Kidney Disease (Open Table in a new window)

Findings

ACKD

ADPKD

Kidney size

Usually not increased; may be decreased because of the advanced renal disease

Increased

Location of cysts

Cortex and medulla

Cortex and medulla

Corticomedullary differentiation*

Possible

Not possible

Normal parenchyma between cysts*

Yes

No

Extrarenal cysts (eg, liver, pancreas)

No

Yes

Positive family history

No

Yes

*On ultrasonography

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Causes

The severity and the duration of azotemia appear to be the critical factors in determining the extent of cyst development.

The causes of multiple renal cysts include the following:

  • Autosomal dominant polycystic kidney disease
  • Autosomal recessive polycystic kidney disease
  • Multicystic renal dysplasia
  • Acquired renal cystic disease
  • Simple renal cysts
  • Medullary sponge kidney
  • Familial juvenile nephronophthisis
  • Medullary cystic disease
  • Von Hippel-Lindau syndrome
  • Tuberous sclerosis complex
  • Glomerulocystic kidney disease
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