Chebib FT, et al. - This review was conducted to describe the approach in evaluating the risk of disease advancement in ADPKD as well as to recognize patients for whom long-term therapy of disease-modifying agents would be beneficial. In ADPKD, it is critical to predict which patient would progress rapidly to renal failure. Clinical, genetic, environmental, epigenetic as well as radiological factors have been studied as predictors of progression to renal failure in ADPKD. Each patient’s unique rate of kidney growth is estimated by age-adjusted total kidney volume (TKV) (represented by the Mayo Imaging Classification), and it affords the most individualized approach available clinically so far. Tolvaptan has gained approval as an agent to slow disease progression in cases at risk of rapidly progressive disease. In clinical trials, various other disease-modifying treatments are being examined. Across countries, there appeared a wide variation in the selection criteria of rapid progression, using an integration of age, baseline GFR, GFR slope, baseline TKV and TKV rate of growth.