From the Guidelines
The treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) should focus on slowing disease progression with Tolvaptan, a vasopressin V2 receptor antagonist, initiated at a dose of 45mg in the morning and 15mg in the afternoon, as recommended by the most recent guideline evidence 1.
Key Considerations
- Tolvaptan has been shown to reduce the decline in eGFR and total kidney volume growth, with a net difference in eGFR decline of 1.3 ml/min per 1.73 m2 per year and in total kidney volume growth of 2.7% 1.
- Patients on Tolvaptan require adequate hydration to replace urinary losses and should have a "sick-day plan" to manage situations that may cause volume depletion 1.
- Blood pressure control is crucial, with a target BP of <120 mm Hg for those aged ≥50 years and/or with CKD G3–G5, and <110/75 mm Hg for people aged 18–49 years with CKD G1–G2 and BP >130/85 mm Hg, using renin–angiotensin system inhibitors as first-line treatment 1.
Lifestyle Modifications
- Maintaining adequate hydration with 2-3 liters of water daily is essential, along with limiting caffeine and sodium intake, and avoiding nephrotoxic medications like NSAIDs.
- A healthy lifestyle, including physical activity and maintenance of normal weight, should be promoted in all patients with ADPKD 1.
Monitoring and Management
- Regular monitoring of kidney function, blood pressure, and screening for complications like intracranial aneurysms is necessary.
- Pain management may involve acetaminophen and sometimes opioids for severe cyst-related pain.
- For end-stage kidney disease, dialysis or transplantation becomes necessary, aiming to preserve kidney function by reducing cyst growth and managing the hypertension that commonly accompanies ADPKD.
From the Research
Treatment Options for Autosomal Dominant Polycystic Kidney Disease (ADPKD)
The treatment for ADPKD has evolved over the years, with various options being explored to slow down the progression of the disease. Some of the key treatment options include:
- Supportive care with blood pressure control and management of pain, urinary infections, and renal stone disease 2
- Use of tolvaptan, which has been approved for use in ADPKD to slow down disease progression 2, 3
- Combination drug therapy, which may include ACE inhibitors, angiotensin receptor blockers, statins, and tolvaptan 3
- Lifestyle modifications, such as optimization of lifestyle and dietary changes 4
Pharmacological Interventions
Pharmacological interventions play a crucial role in the management of ADPKD. Some of the key pharmacological interventions include:
- Tolvaptan, which has been shown to slow down disease progression in patients with rapidly progressive ADPKD 2, 3
- ACE inhibitors and angiotensin receptor blockers, which can help to slow down the progression of kidney disease 3, 4
- Statins, which may be useful in patients with lipid disorders and cardiovascular disease 3
- Mammalian target of rapamycin and dual kinase inhibitors, which are being investigated as potential therapeutic options 5
Non-Pharmacological Interventions
Non-pharmacological interventions also play an important role in the management of ADPKD. Some of the key non-pharmacological interventions include:
- Lifestyle modifications, such as dietary changes and optimization of lifestyle 4
- Management of renal complications, such as cyst infection, nephrolithiasis, haematuria, and chronic pain 4
- Early treatment of patients with ADPKD, which can help to slow down cyst growth and delay subsequent loss of kidney function 4