Kashif J. Piracha, MD, is board-certified in internal medicine and nephrology. He has an active clinical practice at Methodist Willowbrook Hospital in Houston, Texas.
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disease that causes kidney enlargement and fluid-filled cysts to develop on the kidneys. The condition can eventually lead to kidney failure. There is no cure for ADPKD, but it is possible to reduce the number of cysts that form on the kidneys.
If you are diagnosed with ADPKD, you should see a kidney specialist who can help prepare a treatment plan for you. That plan will aim to keep your kidneys functioning and reduce the potential for kidney failure.
Symptoms of ADPKD that are concerning and might require treatment include:
This article will discuss the various treatment options, including medicines available to stop cyst growth and treat various problems linked to ADPKD, lifestyle measures, surgery, and other procedures.
People with ADPKD need to do what they can to keep their kidneys working as long as possible. This includes keeping healthy blood pressure and blood sugar levels, not smoking, and keeping your kidneys and entire body hydrated.
Controlling your blood pressure can delay ADPKD disease progression and slow down kidney damage. You can manage your blood pressure by eating a low-sodium, low-fat diet, not smoking, limiting your alcohol consumption, increasing exercise, and reducing stress.
According to the National Kidney Foundation, diabetes (a condition where blood sugar is high) accounts for 44% of new kidney failure cases. Research shows people with ADPKD can have abnormalities in how their bodies use glucose (sugar) and lipid (fat).
A study reported in 2019 looked at the case records of 189 people with ADPKD. Many had fasting glucose levels of over 100 milligrams per deciliter (a normal fasting blood sugar level is 99 milligrams per deciliter or lower). Seven patients had diabetes, while dyslipidemia (high blood cholesterol) was observed in 30% of the patients and hyperuricemia (high uric levels) in 53%.
Dyslipidemia and hyperuricemia are both risk factors for diabetes, and hyperuricemia is a risk factor for kidney disease.
Even if you have not been diagnosed with diabetes, strict control of blood sugar is important to managing the effects of ADPKD. And lifestyle changes, like diet and exercise, can help prevent diabetic kidney problems early before any kidney damage occurs.
People with ADPKD also have an increased risk for cardiovascular disease (such as stroke, coronary artery disease, and heart attack). If you smoke, your risk increases for these conditions, especially stroke.
A study reported in 2015 sought to understand the effects of smoking on the health outcomes of people with ADPKD. That study included 350 smokers and 371 nonsmokers with ADPKD from 1985 to 2001. In addition, data were collected from surveys during the same time from 159 smokers and 259 nonsmokers with ADPKD.
The researchers found that the smokers with ADPKD had more cardiovascular events, especially strokes, than nonsmoker study participants.
If you smoke, talk to your healthcare provider about quitting. Not only does smoking affect your heart health, but it can also damage the blood vessels of the kidneys and might also promote kidney cyst growth. Quitting smoking is also important for people with ADPKD who have brain aneurysms or a family history of brain bleeds.
A brain aneurysm is a bulge or ballooning of a blood vessel in the brain. About 3%–7% of people with ADPKD will have brain aneurysms. Your risk for an aneurysm with ADPKD increases to up to 15% if someone in your family previously had a brain aneurysm.
Drink plenty of fluids, especially plain water, to keep your kidneys healthy. This is especially important if you notice blood in your urine. Staying hydrated can also reduce the potential for obstructive clots forming in the urinary tract.
It is also wise to avoid excessive caffeine with ADPKD. Studies have found that too much caffeine may encourage faster kidney cyst growth and increased kidney size.
One study reported in 2018 in BMC Nephrology looked at the effect of caffeine on ADPKD. It found that excessive caffeine intake was linked to slightly faster kidney growth, but they didn’t feel the caffeine created a significant detrimental effect on disease progression.
Even though the risk of caffeine consumption in ADPKD seems small, it is still wise to discuss your caffeine intake with your healthcare provider and how it might affect your outlook and disease status.
You might be able to manage and control pain caused by ADPKD with over-the-counter pain relievers that contain acetaminophen, such as Tylenol. If pain is severe, talk to your doctor about stronger prescription pain relief.
Nonsteroidal anti-inflammatory drugs (NSAIDs), such as Advil (ibuprofen) and Aleve (naproxen), are not recommended for people with ADPKD and should be avoided. This is because they can disrupt kidney function and interfere with other treatments, including those used to manage blood pressure.
However, a short course of NSAIDs might be safe for people whose blood pressure is managed and who still have normal kidney function. Talk to your healthcare provider about whether it is safe for you to take NSAIDs for pain relief.
Many different prescription medicines can help manage the effects of ADPKD, including high blood pressure, kidney stones, UTIs, and kidney cyst growth.
If you are experiencing severe pain related to kidney stones or a UTI, treating the underlying cause can bring about relief. However, if the pain is affecting you daily and is severe, your healthcare provider might prescribe a stronger pain reliever, such as codeine or tramadol.
UTIs are generally treated with antibiotics. Tylenol can help to relieve pain or fever. You should see your healthcare provider as soon as possible to treat a UTI to avoid the infection spreading to the cysts in the kidneys. If you experience frequent UTIs, your doctor might prescribe a daily antibiotic in hopes of preventing future UTIs.
If lifestyle changes don’t help to control your blood pressure, your healthcare provider will likely prescribe one or more blood pressure medicines. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) are frequently prescribed to people with ADPKD. These medicines can slow down kidney disease and delay kidney failure.
In 2018, the Food and Drug Administration (FDA) approved Jynarque (tolvaptan), a vasopressin receptor antagonist drug. Jynarque can help slow down cyst growth and preserve kidney function but is not a cure for ADPKD.
Jynarque has also been shown to slow down kidney failure and reduce pain in people with ADPKD. It is recommended for people at high risk for rapid disease progression.
Side effects of Jynarque include liver problems, increased thirst, and frequent urination, especially at night.
Many aspects of ADPKD are manageable with lifestyle changes and medicines. However, some problems that develop in the kidneys or that are considered complications of ADPKD might require surgery or additional procedures.
Small kidney stones (hard deposits made of minerals and salts) will pass out of the body with urination. If a kidney stone is too big to pass naturally, your doctor will recommend a surgical treatment to help remove it.
Procedures to remove or break down kidney stones include:
These procedures are done in the hospital with anesthesia, and most people can go home the same day.
Some cysts can grow very large and cause obstruction or compression of nearby organs or blood vessels. For these cases, your doctor might recommend surgery to drain the cysts.
Infected cysts might also require draining to treat them, especially when aggressive antibiotic therapy isn’t effective.
If you have a family history of ruptured brain aneurysms, your doctor might recommend regular screenings for aneurysms. If an aneurysm is found, surgical clipping can reduce the risk for future rupture or bleeding. In this procedure, a metal clip is placed at the base of the bulge in the artery.
The decision to surgically treat an aneurysm depends on its size. Smaller aneurysms are often treated with the management of high blood pressure and high blood cholesterol and quitting smoking.
If your kidneys lose their ability to remove waste and excess fluid from your body, you will probably need to go on dialysis or get a kidney transplant.
Dialysis is a treatment that performs some of the tasks that your healthy kidneys would normally do. It comes in two types—hemodialysis and peritoneal dialysis.
Hemodialysis is used to rinse the blood and remove excess waste from the bloodstream. With peritoneal dialysis, the rinsing fluid is placed in the abdominal cavity for several hours and then removed.
A kidney transplant is a surgical procedure that takes a healthy kidney from a living or deceased donor and puts it into the person whose kidneys no longer work. It might take years to find a kidney donor match.
Complementary medicine refers to treatments used in addition to standard treatments, and alternative medicine refers to treatments used instead of standard treatments. Complementary and alternative medicine (CAM) is generally not prescribed by kidney specialists to treat ADPKD.
However, certain CAM therapies might help you manage your stress or pain levels. When your stress and pain are reduced, you might experience reduced blood pressure and improved quality of life.
Examples of CAM therapies that can help manage pain and stress are:
A study reported in 2016 in the Journal of Pharmacy and Bioallied Sciences aimed to determine the prevalence of CAM among people with chronic kidney disease (CKD) on maintenance hemodialysis (MHD).
Two hundred people with CKD were asked questions about their CAM knowledge and usage via interview questionnaires adopted from the National Health Interview Survey Adult CAM. Fifty-two of them (26%) were using CAM therapies. The most common were Ayurveda (either alone or with other CAM therapies) and acupuncture.
Because a significant number of people on dialysis are using CAM, study’s authors note the importance of healthcare professionals better informing end-stage kidney disease patients on the risks and benefits of CAM therapies.
Your doctor is the best source of advice on CAM therapies, including their safety and effectiveness, as you live with and manage ADPKD. It is never a good idea to try any CAM therapy without first speaking to your kidney doctor.
Autosomal dominant polycystic kidney disease is an inherited disease that causes kidney enlargement and fluid-filled cysts on the kidneys. Treatment focuses on addressing problems it often creates, including pain, high blood pressure, urinary tract infections, kidney stones, brain bleeds, and over time, kidney failure.
There is no cure for ADPKD but your doctor can recommend lifestyle changes, home remedies, medicines, and different procedures to relieve symptoms and prevent disease complications.
The outlook with autosomal dominant polycystic kidney disease varies from person to person. Some people will experience kidney failure very early in their disease course while others will live the rest of their life with the condition and never need dialysis or a new kidney.
Make sure you keep your doctor updated about any new symptoms or changes to your health. You should also be aware of the serious complications linked to ADPKD, including high blood pressure and brain bleeds. Getting ahead of problems is the best way to improve your outlook and quality of life.
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National Kidney Foundation. Diabetes and chronic kidney disease.
Fliszkiewicz M, Niemczyk M, Kulesza A, et al. Glucose and lipid metabolism abnormalities among patients with autosomal dominant polycystic kidney disease. Kidney Blood Press Res. 2019;44(6):1416-1422. doi:10.1159/000503423
Wang W, Brody JS. Effects of smoking on ADPKD: frequency of vascular events and concentrations of soluble CD40 ligand. General Med. 2015: 3:2 doi:10.4172/2327-5146.100017
National Institute of Diabetes and Digestive and Kidney Diseases. What is polycystic kidney disease?
Bennet WM, Rahbari-Oskoui FF, Chapman AB. Patient education: Polycystic kidney disease (Beyond the Basics). UpToDate.
National Organization for Rare Disorders. Autosomal dominant polycystic kidney disease.
McKenzie KA, El Ters M, Torres VE, et al. Relationship between caffeine intake and autosomal dominant polycystic kidney disease progression: a retrospective analysis using the CRISP cohort. BMC Nephrol. 2018; 19, 378. doi:10.1186/s12882-018-1182-0
Casteleijn NF, Visser FW, Drenth JP, Gevers TJ, Groen GJ, Hogan MC, Gansevoort RT; DIPAK Consortium. A stepwise approach for effective management of chronic pain in autosomal-dominant polycystic kidney disease. Nephrol Dial Transplant. 2014 Sep;29 Suppl 4(Suppl 4):iv142-53. doi:10.1093/ndt/gfu073
OtsukaPharmaceutical Co., Ltd. Otsuka's JYNARQUE™ (tolvaptan) approved by U.S. FDA as the first treatment to slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease (ADPKD).
National Institute of Diabetes and Digestive and Kidney Diseases. Treatment for kidney stones. Updated May 2017.
National Center for Complementary and Integrative Health. Complementary, alternative, or integrative health: What’s in a name?
Arjuna Rao AS, Phaneendra D, Pavani ChD, et al. Usage of complementary and alternative medicine among patients with chronic kidney disease on maintenance hemodialysis. J Pharm Bioallied Sci. 2016;8(1):52-57. doi:10.4103/0975-7406.171692
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