ADPKD-what the radiologist should know.

Area of Science:

• Nephrology
• Medical Imaging
• Pharmacology

Background:

• Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent renal genetic disorder, a leading cause of end-stage renal failure.
• Diagnosis historically relied on imaging, while disease progression monitoring primarily used estimated glomerular filtration rate (eGFR).
• Significant renal parenchyma loss can occur without immediate functional decline, complicating traditional monitoring.

Purpose of the Study:

• To review the pathogenesis, diagnostic challenges, and emerging treatments for ADPKD.
• To highlight the evolving role of imaging in monitoring ADPKD progression.
• To propose MRI-guided volumetric analysis for assessing Tolvaptan's efficacy in slowing renal function decline.

Main Methods:

• Review of current literature on ADPKD pathogenesis and diagnosis.
• Analysis of the role of imaging modalities (sonography, CT, MRI) in disease monitoring.
• Discussion of Tolvaptan's mechanism and clinical impact.
• Proposal for MRI-based volumetric assessment of kidney changes.

Main Results:

• Tolvaptan, a vasopressin antagonist, has demonstrated efficacy in slowing ADPKD progression and preserving renal function.
• Imaging, particularly MRI, is increasingly vital for monitoring disease progression, detecting changes not evident through biochemical markers alone.
• Significant renal volume changes can precede detectable alterations in GFR.

Conclusions:

• ADPKD management requires a multifaceted approach combining understanding of pathogenesis, advanced diagnostics, and novel therapeutics.
• Serial imaging offers a sensitive method for tracking disease progression and treatment response in ADPKD.
• MRI-guided volumetric analysis presents a promising tool for objectively evaluating the effectiveness of treatments like Tolvaptan in ADPKD patients.