Acquired Cystic Kidney Disease

What is acquired cystic kidney disease (ACKD)?

Many people with chronic kidney disease develop ACKD, a condition in which the kidneys develop fluid-filled sacs called renal (kidney) cysts. ACKD occurs in children and adults. The cysts are more likely to develop in people who are on hemodialysis or peritoneal dialysis. Kidney failure, not dialysis, causes the cysts. However, the risk of developing ACKD increases with the number of years a person is on dialysis.

In most cases, the cysts are harmless and require no treatment. Sometimes problems occur-including infection in the cyst, which may be associated with fever and back pain. Sometimes the cysts bleed and blood will appear in the urine. Blood in the urine should always be reported to a doctor.

Although doctors debate the exact percentage, somewhere between 10 and 20 percent of people with ACKD develop kidney tumors, which in some cases are cancerous.2 The rate of kidney cancer in people with ACKD is low, but it is higher than the rate in the general population.

What are the kidneys and what do they do?

The kidneys are two bean-shaped organs, each about the size of a fist. They are located on either side of the spine, just below the rib cage. The kidneys filter wastes and extra fluid from the blood to produce urine. They also release hormones that regulate blood pressure, stimulate the production of red blood cells, and regulate the body's use of calcium to keep the bones healthy.

When the kidneys stop working, a person must receive a new kidney through transplantation or have regular blood-cleansing treatments called dialysis.

What causes ACKD?

Dialysis filters out many, but not all, of the wastes that healthy kidneys remove. Researchers believe that an unidentified waste product not removed through dialysis causes cysts to form in the kidneys. Dialysis itself does not cause the cysts.

How does ACKD differ from polycystic kidney disease (PKD)?

ACKD differs from PKD in several ways. People with PKD often have a family history of PKD. They are born with the disease-causing gene. No disease-causing gene is associated with ACKD. PKD is associated with enlarged kidneys and cyst formation in other parts of the body. In ACKD, the kidneys are normal sized or smaller and cysts do not occur in other parts of the body. In PKD, the presence of cysts marks the onset of disease. People with ACKD already have chronic kidney disease when they develop cysts.

What are the symptoms of ACKD?

ACKD often has no symptoms. If a cyst becomes infected, a person may have back pain, fever, or even chills. If a cyst bleeds, a person will often notice blood in the urine.

How is ACKD diagnosed?

A doctor may suspect ACKD based on a patient's history and symptoms. To confirm the diagnosis, the doctor may order one or more imaging procedures:

Images of the kidneys may help the health care provider distinguish ACKD from PKD.

How is ACKD treated?

If ACKD is not causing pain or discomfort, no treatment is required. Infections are treated with a course of antibiotics. If large cysts are causing pain, they may be drained using a long needle inserted through the skin.

If tumors are suspected, a person may need regular examinations to monitor the kidneys for cancer. Some doctors recommend all patients be screened for kidney cancer after 3 years of dialysis. In rare cases, surgery is used to stop cysts from bleeding and to remove tumors or suspected tumors.

In transplantation, the diseased kidneys are left in place unless they are causing infection or high blood pressure. ACKD usually disappears, even in the diseased kidneys, after a person receives a transplanted kidney.

Points to Remember