Polycystic Kidney Disease (PKD), ARPKD, and ADPKD

Polycystic Kidney Disease (PKD), ARPKD, and ADPKD

What are common complications of ADPKD?

Most people with ADPKD have pain, high blood pressure, and kidney failure at some point in their lives. Common and other complications of ADPKD include:

Pain:  Pain is a common complication of ADPKD and is usually due to kidney or liver cysts. Pain also can be caused by

  • kidney cyst infection,
  • bleeding or burst kidney cysts,
  • urinary tract infection,
  • kidney stones,
  • tissue stretching around the kidney due to cyst growth, and
  • high blood pressure.

High blood pressure:  Almost all people with ADPKD who have kidney failure have high blood pressure. High blood pressure increases your chances of heart disease and stroke. High blood pressure can also damage your kidneys even more. Keep your blood pressure under control to help delay kidney damage.

Kidney failure:  Kidney failure means your kidneys no longer work well enough to stay healthy. Untreated kidney failure can lead to coma and death. More than half of people with ADPKD progress to kidney failure by age 70.

Kidney cancer:  ADPKD complications, or problems, can affect many systems in your body besides your kidneys. Researchers have not found a link between PKD and kidney cancer.

Other complications of ADPKD

  • You may see some ADPKD complications right away.
  • Other complications may not appear for many years, depending on whether you have the PKD1 or PKD2 gene.
  • ADPKD complications can vary from person to person, so you may not have all of these problems.

Vascular system problems

Abnormal heart valves: Abnormal heart valves can occur in some people with ADPKD. Abnormal heart valves can cause too little blood to flow into the aorta, the large artery that carries blood from the heart to the rest of your body. Abnormal heart valves in people with ADPKD rarely need to be replaced. However, you may need more tests if your healthcare provider detects a heart murmur.

Brain aneurysms: An aneurysm is a bulge in the wall of a blood vessel. Aneurysms in the brain might cause headaches that are severe or feel different from other headaches. See a healthcare provider even before you take over-the-counter pain medicines for severe headaches or headaches that won’t go away.

Brain aneurysms can break open and cause bleeding inside the skull. Large brain aneurysms are life-threatening and need immediate medical treatment. If you have an aneurysm, stop smoking and control your blood pressure and lipids.

Digestive system problems

Liver cysts: Liver cysts, which are fluid-filled cysts on the liver, are the most common non kidney complication of ADPKD. Liver cysts don’t usually cause symptoms in people under age 30, because liver cysts are normally small and few in number in the early stages of ADPKD. In rare cases, liver cysts can eventually reduce liver function. In the most severe cases, you may need a liver transplant.

Because the hormone estrogen may affect liver cyst growth, women are more likely to have liver cysts than men. The more pregnancies a woman with ADPKD has had, the more likely she will have liver cysts.

Pancreatic cysts: PKD can also cause cysts in your pancreas. Pancreatic cysts rarely cause pancreatitis, which is inflammation, or swelling, of the pancreas.

Diverticula: Diverticula are small pouches, or sacs, that push through weak spots in your colon wall. Diverticula can cause diverticulosis. Diverticulosis can cause changes in your bowel movement patterns or pain in your abdomen.

Urinary tract problems

Urinary tract infections (UTIs): Kidney cysts can block urine flow through the kidneys so that urine stays in your urinary tract too long. When urine stays in your urinary tract too long, bacteria in your urine can cause a bladder infection or a kidney infection. A kidney infection can cause further damage to your kidneys by causing cysts to become infected.

Kidney stones: People with ADPKD sometimes have kidney stones. Kidney stones can block urine flow and cause infection and pain.

Reproductive problems

Although most women with PKD have normal pregnancies, women with PKD who have high blood pressure and decreased kidney function are more likely to have preeclampsia or high blood pressure during pregnancy.

With preeclampsia, the fetus gets less oxygen and fewer nutrients. Women with preeclampsia should be followed closely by their health care provider during and after pregnancy. After delivery, preeclampsia goes away.

Many men with ADPKD have cysts on their seminal vesicles, which are glands in the male reproductive system that help produce semen. Seminal vesicle cysts rarely cause infertility.

People with PKD who are considering having children may want to discuss family planning concerns with a genetics counselor.