Cystic Kidney Disease

What is cystic kidney disease?

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

Another name for cystic kidney disease is renal cystic disease.

How common is cystic kidney disease?

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

What are the symptoms of cystic kidney disease?

The various cystic kidney diseases have different symptoms. But some of the most common symptoms include:

• Back pain
• Flank pain
• Blood in your pee (hematuria)
• Trouble peeing, including not peeing as much as you usually do
• Enlarged kidneys or kidney masses
• Headaches
• High blood pressure
• Kidney infections
• Kidney stones

What causes cystic kidney disease?

Cystic kidney diseases have different causes. Some may result from genetic variations. Others might develop over time due to diseases, birth defects or age.

Cysts occur when renal tubule pieces detach from a larger parent tube. Your kidneys have thousands of tiny tubes that clean your blood and release pee into your bladder.

Who does cystic kidney disease affect?

Risk factors for cystic kidney disease vary widely across the different types. But in general, you’re more likely to get cystic kidney disease if you’re:

• 50 or older
• Have chronic kidney disease (CKD) or kidney failure
• Have an abnormal gene

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 if you have polycystic kidney disease)
• Liver cysts and pancreatic cysts (more common if you have polycystic kidney disease)
• Problems with growth and development delays in infants

How is cystic kidney disease diagnosed?

A healthcare provider will ask you about your symptoms and review your medical history. They’ll also order one or more of the following imaging tests to check for kidney cysts:

• Prenatal ultrasound or kidney ultrasound
• CT scan
• MRI

A provider will also likely order blood tests and a pee test (urinalysis) to see how well your kidneys are filtering your blood.

Is cystic kidney disease curable?

Simple kidney cysts that don’t cause any symptoms may not need treatment. A healthcare provider may monitor the cysts and perform annual ultrasounds to make sure they don’t grow. If the cysts are painful or cause other symptoms, they may perform fine-needle aspiration to drain the cyst or laparoscopic surgery to cut or burn away the cyst tissue.

If you develop kidney failure from cystic kidney disease, a provider may recommend:

• Dialysis to clean your blood when your kidneys no longer work
• Kidney transplant (you may get a kidney from a living or deceased organ donor)
• Blood pressure medications (antihypertensives) to manage high blood pressure
• Lifestyle changes to manage high blood pressure, including at least 30 minutes of activity per day, maintaining a healthy weight for you and avoiding tobacco products, including smoking

Should a polycystic kidney be removed?

It depends. Unless you’re in a lot of pain or have other symptoms, healthcare providers usually don’t recommend removing your kidneys, even if they stop filtering wastes. They may still filter excess water from your body. But a provider may recommend a nephrectomy to remove your damaged kidneys if they cause a lot of pain or other symptoms.

How can I prevent cystic kidney disease?

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

What can I expect if I have 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. You may need dialysis or a kidney transplant.

At what age do people with PKD go into kidney failure?

This can vary. It depends on the specific genetic variation. But it often has a similar pattern in a given family. PKD (polycystic kidney disease) involves two major genes — PKD1 and PKD2. People with the PKD1 gene variation tend to go on to kidney failure sooner (around mid-50s) than people with the PKD2 gene variation (early 70s).

Your healthcare team will give you a better idea of what to expect according to your unique situation.

What is the life expectancy of someone with polycystic kidney disease?

Nearly 80% of people with autosomal dominant polycystic kidney disease have preserved kidney function at 50. Over 50% have preserved kidney function into their early 70s.

About one-third of babies who have autosomal recessive polycystic kidney disease at birth don’t survive. Babies who do survive need treatment for the rest of their lives.

What foods should I avoid with kidney cysts?

You may need to work with a renal dietitian to develop kidney-friendly eating patterns if you have chronic kidney disease or reduced kidney function due to cystic kidney disease. This may include:

• Avoiding foods high in sodium (salt)
• Limiting the amount of protein you eat
• Eating heart-healthy foods
• Avoiding foods that have high amounts of the electrolytes phosphorus and potassium, including dairy, beans, bananas, oranges and potatoes

When should I see a healthcare provider?

Call a healthcare provider or get to the nearest emergency room if you have the following signs of sudden kidney failure, including:

• Abdominal pain
• Trouble peeing, including not peeing as much as you typically do
• Swelling (edema), especially around your hands, ankles or face
• Feeling very tired (fatigue) or drowsy
• Itchy skin
• Loss of appetite
• Nausea and vomiting
• Shortness of breath

What questions should I ask a healthcare provider?

You may want to ask your provider:

• How will you diagnose cystic kidney disease?
• Does cystic kidney disease affect one or both kidneys?
• Do I need treatment?
• What treatment do you recommend?
• How can I help take care of my kidneys?
• Can you recommend a nephrologist or renal dietitian?
• Can you recommend any cystic kidney disease support groups?

Is a cyst in the kidney serious?

It depends. Simple kidney cysts don’t make your kidneys larger, replace kidney tissue or affect how well your kidneys work. They usually don’t develop into a serious problem.

Complex kidney cysts can affect the size, structure and function of your kidneys. They can also be a sign of kidney cancer.

If you have a kidney cyst, healthcare providers will monitor it for changes and recommend treatment, if necessary.

Are there different types of cystic kidney disease?

Yes, there are several types of cystic kidney disease. Some types are genetic. That means they develop because of variations in certain genes in your body. If you have a genetic kidney disease, you usually inherit the gene variations from one or both of your biological parents. A genetic variation can also start with you. Other types of cystic kidney disease may develop during your lifetime (acquired).

What are some genetic cystic kidney diseases?

Some genetic cystic kidney diseases include:

• Polycystic kidney disease (PKD). PKD causes cysts that enlarge your kidneys and affect how they work by crowding out the filtering units of your kidney.
• Glomerulocystic kidney disease (GCKD). GCKD is an autosomal dominant condition (only one biological parent needs to have the genetic variation to pass it on to their child) that causes cysts to develop. It also causes the space in your kidneys near your urinary system to get bigger.
• Medullary cystic kidney disease (MCKD). MCKD is an autosomal dominant condition that causes cysts to develop in the inner (corticomedullary) part of your kidneys. It leads to inflammation and scarring of your renal tubules, which help your kidneys filter waste.
• Nephronophthisis. Nephronophthisis is an autosomal recessive condition that’s 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?

• Simple kidney cysts. These types of cysts are common and typically affect people 50 and over. In most cases, they don’t harm your kidneys. But in rare cases, they can transform into complex cysts that require monitoring because they have the potential to become cancerous (malignant).
• Acquired cystic kidney disease. This disease causes cysts to develop over time due to chronic kidney disease (CKD) or kidney failure. It tends to affect people on dialysis.
• Multicystic dysplastic kidney. Multicystic dysplastic kidney is when one or both kidneys don’t develop properly in the uterus. Cysts cover most of one or both of your kidneys.
• Medullary sponge kidney. This is a rare condition in which cysts form on the innermost part of your kidneys and block your renal tubules.