Since 2017, tolvaptan has been available to slow disease progression, delaying end-stage renal failure by about one year after four years of treatment, though it causes “aquaretic” side effects such as high urine output, thirst, and nocturia.
Long-term tolvaptan therapy reduces kidney function decline by up to 24% compared with untreated controls, with stronger effects in patients showing greater vasopressin blockade. Dietary reduction of salt and protein modestly decreases urine volume by 11%, especially in those with high baseline output.
A restrictive approach to pre-transplant nephrectomy is supported: only about one-third of patients require kidney removal before transplantation, with no difference in survival or surgical complications compared to post-transplant procedures. Quality of life improves after transplantation and nephrectomy regardless of timing, and bilateral nephrectomy yields better physical outcomes than unilateral removal.
A systematic review and collaboration with European kidney and urology societies produced a guideline recommending routine imaging to assess kidney size, shared decision-making, a preference for unilateral minimally invasive surgery when space is limited, and the practice point that prophylactic nephrectomy before transplantation is unnecessary.
Finally, a stepwise, multidisciplinary pain management protocol (from non-pharmacologic to surgical interventions) effectively reduced pain and opioid use in patients with chronic kidney-related pain, demonstrating broader applicability beyond ADPKD.
