Cystic Kidney Disease

What is cystic kidney disease?

Cystic kidney disease (CKD) describes a group of conditions that cause cysts (fluid-filled sacs) to form in or around the kidneys. Kidney cysts can prevent the kidneys from filtering water and waste out of your blood. Cystic kidney disease can lead to kidney failure.

Are there different types of cystic kidney disease?

There are several types of CKD. Some are the result of mutations (changes) to certain genes that are usually inherited (passed from parents to children). Others may develop during a person’s lifetime, or they might be congenital (present at birth). Cysts can also appear in the kidney later in life.

What are some genetic cystic kidney diseases?

Genetic cystic kidney diseases include:

• Polycystic kidney disease (PKD): Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of cystic kidney disease in adults. It’s usually diagnosed between the ages of 30 and 50. Another type of PKD is autosomal recessive polycystic kidney disease (ARPKD). It causes abnormal kidney development in the uterus or soon after birth. ARPKD is rare.
• Glomerulocystic kidney disease (GCKD): GCKD causes cysts and enlargement of the space in the kidneys near the urinary tract. It’s very uncommon but can affect infants or adults.
• Medullary cystic kidney disease (MCKD): MCKD causes cysts to develop in the corticomedullary (inner) part of the kidneys. It leads to inflammation and scarring of the tubes that help the kidneys filter waste.
• Nephronophthisis: This condition is very similar to MCKD, but it affects infants, children and teenagers. It usually leads to kidney failure before adulthood.

What are some non-genetic cystic kidney diseases?

Non-genetic cystic kidney diseases include:

• Simple kidney cysts: These cysts are fairly common and usually affect older individuals. They don’t tend to enlarge or disrupt the kidneys. In rare cases, cysts can be malignant (cancerous) and lead to kidney cancer.
• Acquired cystic kidney disease: This disease causes cysts to develop over time due to chronic kidney disease or kidney failure. It tends to affect adults or children who are on dialysis.
• Multicystic dysplastic kidney: Kidney dysplasia is a common condition that occurs when one or both kidneys don’t develop correctly in the uterus. Cysts replace normal kidney tissue.
• Medullary sponge kidney: Cysts form on the innermost part of the kidneys. They block the tubes that filter urine. It’s rare.

Who is at risk of developing cystic kidney disease?

Risk factors for CKD vary widely across the different types. In general, you’re more likely to get it if you:

• Are older.
• Have chronic kidney disease or kidney failure.
• Have an abnormal gene.

How common is cystic kidney disease?

Some cystic kidney diseases are very common. For instance, simple kidney cysts occur in about 1 out of 10 people. But other forms of the disease are very rare.

What causes cystic kidney disease?

Cystic kidney diseases can be the result of genetic mutations. Or they might develop over time due to diseases, birth defects or age. The cysts themselves occur when pieces of the renal tubes detach from the parent tube. The kidneys have thousands of tiny tubes that clean your blood and release urine into the bladder.

What are the symptoms of cystic kidney disease?

The various cystic kidney diseases have different symptoms. Some of the most common among them include:

• Back or flank pain (pain in your back or sides).
• Burst or bleeding cysts.
• Difficulty urinating, or not producing much urine.
• Enlarged kidneys (more common in PKD) or kidney masses.
• Headaches.
• Hematuria (blood in the urine).
• High blood pressure.
• Kidney infections.
• Kidney stones.

What are the complications of cystic kidney disease?

Some of the more common complications of the various cystic kidney diseases include:

• Kidney failure.
• Heart valve problems (more common in PKD).
• Liver cysts and pancreatic cysts (more common in PKD).
• Problems with growth and development in infants.

How is cystic kidney disease diagnosed?

Your healthcare provider evaluates your symptoms and medical history. They may do one or more of the following imaging tests to check for kidney cysts:

• Kidney ultrasound or prenatal ultrasound.
• CT scan.
• MRI.
• Blood and urine tests can also give your healthcare provider important information about how well your kidneys are working and filtering your blood.

How is cystic kidney disease treated?

Simple kidney cysts that aren’t causing symptoms may not need treatment. Your healthcare provider might monitor the cysts and do ultrasounds to make sure they don’t grow or spread. If cysts are causing symptoms, your healthcare provider may be able to drain or remove the cysts.

Treatment for more complex forms of cystic kidney disease may include:

• Dialysis, which is a procedure to clean your blood when the kidneys fail.
• Kidney transplant for people who are in kidney failure.
• Medication and lifestyle changes to manage your blood pressure.

How can I prevent cystic kidney disease?

There’s no way to prevent CKD. But talking to your healthcare provider when you first notice symptoms may help slow the progression of some forms of cystic kidney disease.

What is the outlook for people with cystic kidney disease?

There’s no cure for cystic kidney disease, but there are many treatment options to slow the progression of polycystic kidney disease. Most people can lead full lives, but may need dialysis or a kidney transplant.

When should I call my doctor about cystic kidney disease?

Call your doctor right away if you experience any of the following symptoms. These could be signs of sudden kidney failure:

• Abdominal pain.
• Difficulty urinating, or not producing much urine.
• Edema (fluid buildup and swelling in your legs or feet).
• Fatigue or drowsiness.
• Itchy skin.
• Loss of appetite.
• Nausea and vomiting.
• Shortness of breath.

A note from Cleveland Clinic

Cystic kidney disease describes a group of disorders that cause cysts to form in or around your kidneys. Some people inherit CKD through abnormal genes; others develop CKD over time. Sometimes kidney cysts are harmless. But certain forms of cystic kidney disease (such as polycystic kidney disease) can prevent your kidneys from working properly, resulting in serious health problems. People with CKD may need medication to manage complications such as high blood pressure, dialysis or a kidney transplant.