. 2017 Mar 1;4:2054358117695784. doi: 10.1177/2054358117695784

Table 4.

Prognostic Factors Related to Disease Progression in ADPKD.

Imaging-based prognostic factors

● TKV shows a strong inverse association with the slope of GFR^28,41

● Height-adjusted TKV shows a good correlation with GFR at baseline (r = 0.22), and an even stronger correlation after 3 years and 8 years (r = 0.44 and r = 0.65, respectively)⁴²

Genetic prognostic factors

● PKD1 mutations are associated with an earlier onset of ESRD compared with PKD2 mutations⁴³

● Truncating PKD1 mutations are associated with an average onset of ESRD at 55 years of age, whereas nontruncating PKD1 mutations and PKD2 mutations are associated with an average onset of ESRD at 67 and 79 years of age, respectively⁴⁴

● Hypomorphic alleles are associated with milder disease as polycystin activity is not completely abrogated; if these alleles are coupled with another mutation, a more severe disease progression may develop⁴⁵

Urinary biomarkers

● Urinary neutrophil gelatinase–associated lipocalin and interleukin-18 levels increased over 3 years in the CRISP study; however, the increases in these 2 urine biomarkers did not correlate with changes in TKV or kidney function⁴⁶

Other prognostic factors

● Higher levels of vasopressin activity (measured using 24-h urine osmolality as a surrogate marker) were associated with greater declines in GFR from year 1 to 6 in the CRISP study⁴⁷

● Increased vasopressin activity (measured using copeptin levels as a surrogate marker) was associated with higher morning urine osmolality, higher BP, increased TKV, and decreased GFR in the CRISP study⁴⁸

● Elevated serum uric acid levels are associated with disease progression; a 5.8% increase in TKV and a 4.1% increase in TKV/body surface area for every 1-mg/dL increase in uric acid have been demonstrated⁴⁹

Note. ADPKD = autosomal dominant polycystic kidney disease; TKV = total kidney volume; GFR = glomerular filtration rate; ESRD = end-stage renal disease; CRISP = Consortium of Renal Imaging Studies in Polycystic Kidney Disease; BP = blood pressure.