Among 227 ADPKD patients with an identified pathogenic or likely pathogenic variant in either PKD1 or PKD2, 38 of 171 (22%) with a PKD1 mutation had pleural fluid compared to 9 of 56 (16%) with a PKD2 mutation (p = 0.32) (Table 3). Here, PKD2 is linked to autosomal dominant polycystic kidney disease.