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Treating Autosomal Dominant Polycystic Kidney Disease

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Polycystic Kidney Disease: 9 Things Doctors Want You to Know

Doctor William C Lloyd Healthgrades Medical Reviewer
Medically Reviewed By William C. Lloyd III, MD, FACS
Written By Marijke Vroomen Durning, RN on April 17, 2020
  • doctor-looking-at-liver-scan
    Expert Advice About Polycystic Kidney Disease, Also Called PKD
    Polycystic kidney disease (PKD) is a common genetic disease where noncancerous cysts form within the kidney causing it to enlarge and affecting kidney function. Two nephrologists—kidney specialists who treat PKD and other kidney disorders—discuss issues surrounding PKD, what it is, who may get it, special diet requirements for someone who has PKD, the prognosis, and more.
  • Portrait of happy daughter embracing senior woman
    “Polycystic kidney disease is the most common genetic disease in the U.S.”
    According to the National Kidney Foundation, polycystic kidney disease affects about 600,000 people in the United States and it’s the fourth leading cause of kidney failure. It’s a genetic disease, which means PKD is passed from parent to child. “It affects about one in 250 or 300 births,” explained Robert C. Greenwell, MD, the chief of nephrology at Mercy Medical Center, Baltimore. “And it only takes one of the abnormal genes from either parent for PKD to be passed on,” he added.
  • Scientist holding a tube containing a blood sample ready for analysis in the laboratory
    “Genetic testing for PKD is not routine.”
    “People who are diagnosed with polycystic kidney disease may be just at the stage of starting their family, and might be concerned about passing on the disease,” said Amanda C. Raff, MD, a nephrologist, at the Montefiore Health System in New York City. But genetic testing is usually only done in specific situations. “If you're considering being a [kidney] donor for your parent, for example, it's absolutely essential to know whether or not you're carrying the mutation,” Raff continued. “They wouldn't accept you as a donor if you're carrying the mutation because you would be at risk for kidney disease yourself.”
  • Senior couple dancing in living room
    “Polycystic kidney disease patients often do quite well for a long time.”
    Being told you have a chronic disease that could shorten your life can be frightening, but Greenwell assures his patients that usually there is a long period of time between the diagnosis and when the kidney problems may begin to surface. “I always tell my patients, whether you've got this or not, whether it progresses to you needing kidney dialysis or kidney transplant, those are things you might have to deal with, but any of that stuff is not going to stop you from getting to whatever plans you have down the road.”
  • Happy friends enjoying Christmas party at home
    “Most people don’t notice any discomfort of symptoms for quite a while.”
    Although PKD causes your kidneys to grow unusually large, most people don’t notice any discomfort from this, said Greenwell. “It’s mostly an asymptomatic [no symptoms] issue that periodically can cause issues,” he explained. They may see blood in their urine, for example. Patients with PKD are also at a bit higher risk for kidney stones. Greenwell said that about half his PKD patients initially see him because they have a family history of kidney disease and they want to know if they have it too.
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  • Male patient and doctor in discussion in exam room
    “There are two types of polycystic kidney disease.”
    There are two types of polycystic kidney disease. “One gene tends to have a later onset of disease and then the other, but there's a lot of variability,” said Raff. About 90% of people with polycystic kidney disease have autosomal dominant PKD, or ADPKD. This form tends to be diagnosed among people between the ages of 30 and 50 years old. The remaining have autosomal recessive PKD, or ARPKD, which can affect people as early as infancy.
  • Young African American pregnant woman getting blood pressure checked by African American female doctor
    “PKD can cause complications to other organs.”
    Although polycystic kidney disease directly affects the kidneys, any type of kidney disease can cause complications to other organs. “Patients with polycystic kidney disease often develop high blood pressure in the early stage of the disease, even before there’s any change in kidney function,” explained Raff. “It’s important to have it aggressively treated.” Other complications can include cysts in the liver and pancreas, issues with the heart valves or colon, and pregnant women are at higher risk for preeclampsia.
  • Dialysis nurse checking dialysis machine
    “There is no cure for polycystic kidney disease.”
    There is no cure for PKD. Treatment focuses on keeping the kidneys as healthy as possible and treating problems that arise with other organs. A new medication, Tolvaptan, was approved by the FDA in 2018, to slow down kidney decline. “It's an antidiuretic, so you make less urine because you're holding onto more water,” explained Raff. “It’s the first time there's been a medication that's approved and [has] shown efficacy for PKD.” Careful monitoring of blood tests tells your nephrologist how well your kidneys are functioning. Once the function is too low, treatment is regular dialysis, and possibly a kidney transplant.
  • Colorful healthy vegan meal in salad bowl
    “People with PKD must avoid consuming certain things.”
    Medications that are broken down in the kidney, such as nonsteroidal anti-inflammatories (NSAIDs) must be avoided if you have PKD. “Those are medications that are very common in use over the counter and are harmful for people [with PKD],” said Raff. Speak with your pharmacists before taking any drug, supplement or vitamin. People with PKD should speak with a dietitian about a polycystic kidney disease diet—foods that are good for them and those they should avoid, particularly if they have high blood pressure. General guidelines include consuming a low-fat diet with lots of fruit and vegetables, and limiting alcohol.
  • Senior couple tourist with bicycles cycling in town on holiday
    “People with PKD can live a long time.”
    People may be diagnosed with polycystic kidney disease when they’re in their 30s, 40s, or 50s, but that doesn’t mean they’ll have any limitations for many years to come. “Some patients never even reach kidney failure and need dialysis,” said Greenwell. “And, even the ones who will reach that often do continue to live a very normal life even with dialysis. I like to tell them that the diagnosis might affect how their kidneys will be, but it really doesn't change the fact that that they'll still be able to do and plan all the things they want to do in the years to come.”
Polycystic Kidney Disease: 9 Things Doctors Want You to Know
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Last Review Date: 2020 Mar 31
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