Canada's kidney disease specialists have released a comprehensive roadmap for improving care for people with autosomal dominant polycystic kidney disease (ADPKD), the most common form of inherited polycystic kidney disease (PKD). The Canadian Society of Nephrology workgroup reviewed the 2025 KDIGO (Kidney Disease: Improving Global Outcomes) guideline and adapted it specifically for Canada's healthcare system, highlighting gaps in access, coordination, and specialized services that affect patients across the country.

For many people living with ADPKD, navigating kidney care feels fragmented. A patient might see a family doctor, nephrologist, radiologist, genetic counselor, transplant team, liver specialist, pain clinic, or obstetrics team, but these services often operate independently. The new Canadian commentary emphasizes that this scattered approach leaves patients responsible for piecing together their own care plan, which can delay diagnosis, miss important complications, and prevent timely treatment decisions.

Why Does ADPKD Require Such Specialized, Coordinated Care?

ADPKD is far more complex than kidney cysts alone. The disease can affect multiple body systems and create a cascade of interconnected health challenges. The Canadian workgroup identified the full scope of complications that require expert coordination:

• Kidney-Related Issues: Progressive kidney function decline, high blood pressure that often appears early, chronic pain from enlarged kidneys, kidney stones, and cyst infections requiring specialized management.
• Liver Involvement: Polycystic liver disease with cysts that can grow large enough to cause symptoms, requiring hepatology expertise and sometimes medication management.
• Cardiovascular and Neurological Risks: Brain aneurysm risk that may require screening and neurosurgical consultation, plus heart health concerns linked to early high blood pressure.
• Reproductive and Family Planning: Pregnancy complications, genetic counseling for family members, and decisions about genetic testing for children or relatives who may inherit the condition.
• Mental Health and Psychosocial Support: The emotional burden of managing a lifelong genetic disease, planning for dialysis or transplant, and coping with uncertainty about disease progression.

What Are the Major Gaps in Canadian PKD Care Today?

The commentary identifies several structural barriers that prevent people with ADPKD from receiving timely, comprehensive care. Long wait times for specialist appointments, uneven access to genetic testing across provinces, limited availability of expert clinics in rural and remote regions, and inconsistent drug coverage for ADPKD-specific medications create a patchwork system where geography and provincial residence determine access to care. Indigenous communities face additional barriers to specialized nephrology services and genetic counseling.

The workgroup also noted that imaging access is not equal across Canada. Magnetic resonance imaging (MRI), which is the gold standard for assessing kidney size and predicting disease progression using the Mayo Imaging Classification, is not uniformly available. This matters because kidney size is one of the strongest predictors of how quickly ADPKD will progress, and this information should guide treatment decisions, including whether a patient might benefit from tolvaptan, the only approved ADPKD-specific medication that targets cyst growth.

How Should Canadian ADPKD Care Be Reorganized?

The Canadian Society of Nephrology workgroup recommends building stronger multidisciplinary ADPKD networks that connect nephrology, genetics, radiology, hepatology, urology, neurosurgery, transplant teams, pain specialists, dietitians, nurses, pharmacists, social workers, physiotherapists, and patient support groups. This coordinated approach would ensure that patients receive comprehensive evaluation and that specialists communicate about shared patients.

One of the most ambitious recommendations is the creation of a national ADPKD registry. This registry would help Canada understand how many people have ADPKD, identify where care gaps exist, track how the disease affects different communities, and measure how treatments are working over time. The workgroup also suggests developing a Canadian list or map of ADPKD centers of excellence and available services, so patients and clinicians know where to find expert care, including virtual consultation options for people in remote areas.

What Specific Changes Are Recommended for Key Areas of ADPKD Care?

Genetic Testing and Counseling: The commentary calls for clearer pathways so patients and clinicians understand when genetic testing is useful, how to access it, and who can explain results. Genetic testing becomes especially important when the diagnosis is unclear, there is no known family history, a living related donor is being considered for transplant, or a child or young person may be affected. Canada's Genetic Non-Discrimination Act provides some protection against discrimination based on genetic test results, though gaps remain for people with existing kidney disease or known family history.

Blood Pressure Management: The commentary supports careful, standardized blood pressure measurement, including home or ambulatory blood pressure monitoring when possible. Blood pressure control is critical in ADPKD because high blood pressure often appears early and can accelerate kidney and heart damage. The workgroup recommends reducing financial barriers to home blood pressure monitors and 24-hour blood pressure testing.

Chronic Pain Management: Some people with ADPKD live with serious pain from enlarged kidneys, cysts, stones, infections, or other causes. The commentary recommends a step-by-step approach: start with pain management at the local level, bring in specialists when needed, and refer complex cases to centers with ADPKD expertise.

Transplant Planning: The workgroup emphasizes that removing native PKD kidneys before transplant should not be automatic. Nephrectomy decisions should be shared between the patient, nephrologist, and surgeon. Pre-transplant nephrectomy is generally considered only when kidneys are very large and create space problems or when symptoms are severe, because removing one or both kidneys carries risks, including needing dialysis sooner.

Dialysis Options: The commentary clearly states that ADPKD should not automatically rule out peritoneal dialysis, a home-based dialysis method. While large kidneys and hernia risk may require special consideration, peritoneal dialysis can be a viable option for some people with ADPKD.

What Does the Commentary Say About ADPKD Medications and Treatments?

Tolvaptan is confirmed as the only approved ADPKD-specific treatment that targets cyst growth. However, the workgroup emphasizes that treatment decisions should be individualized, taking into account risk level, kidney size, rate of kidney function decline, family history, age, side effects, thirst, frequent urination, and personal goals. A significant barrier is that tolvaptan funding varies across Canada, creating unequal access to this therapy.

For newer chronic kidney disease (CKD) drugs like SGLT2 inhibitors, the commentary is cautious. These medications have helped many people with kidney disease, but people with ADPKD were often excluded from major clinical trials. The workgroup says SGLT2 inhibitors should not be used specifically to slow ADPKD progression at this time, though they may still be considered for other reasons, such as diabetes or heart failure, depending on the patient.

For ketogenic diets and ketone supplements, the commentary states there is not enough human evidence to recommend them for ADPKD. The authors also raise safety concerns, including cholesterol changes, kidney stones, and ketoacidosis in people with lower kidney function.

What About Liver Cysts, Brain Aneurysms, and Pregnancy Planning?

For polycystic liver disease, the commentary notes that some medications called somatostatin analogues may help reduce liver volume in certain people with severe symptoms. However, access, cost, specialist experience, and liver volume measurement are all barriers in Canada. The workgroup suggests that regional centers of expertise could help manage severe liver cyst disease more consistently.

For brain aneurysm screening, the KDIGO guideline recommends screening for people with ADPKD who have a personal or family history of aneurysm, brain bleed, or unexplained sudden death, if they would be candidates for treatment. The Canadian workgroup also suggests that other patients may benefit from discussing screening, even without a known family history, using shared decision-making.

For pregnancy and reproductive care, the commentary emphasizes that Canada needs better preconception counseling for people with ADPKD. These conversations should cover blood pressure, kidney function, protein in the urine, aneurysm screening, liver cysts, genetic testing, preimplantation genetic testing, and high-risk pregnancy care. Access to these services is currently uneven and often costly.

For children and youth with ADPKD, the commentary supports blood pressure monitoring after age 5 and better transition programs from pediatric to adult care. Care should be flexible, because every child and family situation is different.

This Canadian commentary represents a major step forward for PKD care in the country. By calling for earlier diagnosis, better risk assessment, fairer access to treatment, and coordinated multidisciplinary networks, the workgroup has outlined a path toward more equitable and effective ADPKD management across all provinces and communities.