Autosomal dominant polycystic kidney disease (
ADPKD) is one of the most common
genetic diseases with a reported prevalence of 1:400 to 1:1000. Since the intact kidneys can compensate for the loss of glomerular filtration in
ADPKD patients,
renal insufficiency usually remains undetected until almost the fourth decade of life. Hereafter, reliable diagnostic and prognostic
biomarkers to identify
ADPKD progression are urgently needed. Several studies and systematic reviews tried to identify markers or predictors of rapid
disease progression of
ADPKD. The aim of this study is to review predictors of rapid
disease progression of
ADPKD that can be useful to the clinician. We will describe several factors associated with rapid progression of
ADPKD derived from retrospective or cross-sectional studies, suggesting the best and most useful predictors that may help to patients management in clinical practice. We will attempt to identify the most useful predictors of rapid
disease progression of
ADPKD: established TKV growth rate >5% per year, annual estimated glomerular filtration rate decline >5 mL/min/1.73 m2, truncating PKD1 mutations and elevated plasma copeptin level. The combination of several factors that can predict the rapid
ADPKD progression is more accurate than a single-marker strategy. The "PRO-PKD" risk scoring system combined with TKV, can be useful in order to evaluate the
ADPKD patients and they appear to be appropriate predictors of
progression disease. Moreover levels of copeptin and some urinary markers can be matched to these factors for improved patient assessment in rapid progression.