From the Guidelines
Polycystic kidney disease (PKD) prognosis is highly variable, but approximately 50% of patients with autosomal dominant PKD will develop end-stage renal disease by age 60, and treatment should focus on slowing disease progression through blood pressure control, lifestyle modifications, and the use of tolvaptan in appropriate candidates, as recommended by the KDIGO 2025 clinical practice guideline 1.
Factors Affecting Prognosis
The prognosis of PKD depends on several factors, including:
- Genetics: PKD1 mutations progress faster than PKD2 mutations 1
- Gender: Males typically progress faster than females
- Blood pressure control: Hypertension can accelerate disease progression
- Kidney size: Larger kidneys are associated with a worse prognosis
- Presence of complications: Infections, kidney stones, and other complications can impact prognosis
Diagnosis and Management
Diagnosis of PKD typically involves imaging studies, such as ultrasound or MRI, and genetic testing may be helpful in some cases 1. The Mayo Imaging Classification (MIC) can be used to predict future decline in kidney function and the timing of kidney failure 1.
Treatment
Treatment of PKD should focus on:
- Blood pressure control with ACE inhibitors or ARBs
- Pain management
- Treating complications like infections and kidney stones
- Lifestyle modifications, including adequate hydration, low-sodium diet, and avoiding nephrotoxic medications
- Use of tolvaptan, a vasopressin receptor antagonist, in appropriate candidates to slow kidney function decline 1
Monitoring and Screening
Regular monitoring of kidney function, blood pressure, and screening for complications is essential to manage PKD effectively. This includes:
- Monitoring kidney function with eGFR and urine albumin-to-creatinine ratio
- Screening for complications like liver cysts, brain aneurysms, heart valve abnormalities, and diverticular disease
- Genetic counseling for family planning, as PKD has a 50% inheritance risk for children of affected individuals 1
From the Research
Polycystic Kidney Disease Prognosis
- The prognosis for patients with polycystic kidney disease (PKD) varies depending on several factors, including the type of PKD, the severity of the disease, and the presence of other health conditions 2.
- Autosomal dominant polycystic kidney disease (ADPKD) is a common form of PKD that typically presents in adults, while autosomal recessive polycystic kidney disease (ARPKD) is a rarer and often more severe form that usually presents in children 2.
- Studies have shown that patients with PKD who receive a kidney transplant have better long-term graft and patient survival compared to those with other native diseases 3.
- The use of angiotensin-converting enzyme (ACE) inhibitors, such as lisinopril, has been shown to be beneficial in reducing the progression of renal cystogenesis in PKD patients 4, 5.
- However, ACE inhibitors may worsen renal function in patients with ADPKD and massive renal involvement, and caution is recommended when using these medications in such patients 4.
- Dual blockade of the renin-angiotensin-aldosterone system (RAAS) with an ACE inhibitor and an angiotensin II-receptor blocker (ARB) has been shown to be effective in controlling blood pressure and reducing urinary aldosterone excretion in patients with ADPKD, but it does not alter the decline in estimated glomerular filtration rate (GFR) 6.
Factors Affecting Prognosis
- The presence of other health conditions, such as coronary artery disease and higher body mass index (BMI), can affect the prognosis of patients with PKD 3.
- The use of calcineurin inhibitors (CNIs) has been associated with a lower risk of death-censored graft failure (DCGF) in patients with PKD who receive a kidney transplant 3.
- HLA mismatch and pretransplant coronary artery disease have been associated with a higher risk of DCGF and death in patients with PKD who receive a kidney transplant 3.