Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common inherited disorders. It is the fourth leading cause of renal replacement and renal failure worldwide. Mutations in PKD1 or PKD2 cause ADPKD. Patients with ADPKD show progressive growth of renal cysts filled with cystic fluid, leading to end-stage renal disease (ESRD) and renal failure by their sixth decade of life. Currently, there are no curative treatments for ADPKD. Therefore, patients require dialysis or kidney transplantation. To date, researchers have elucidated many of the mechanisms that cause ADPKD and developed many methods to diagnose the disease. ADPKD is related to growth factors, signaling pathways, cell proliferation, apoptosis, inflammation, the immune system, structural abnormalities, epigenetic mechanisms, microRNAs, and so on. Various therapies have been reported to slow the progression of ADPKD and alleviate its symptoms.
Keywords: ADPKD; Cyst; Disease mechanism; ESRD; Pathogenesis; Polycystic kidney; Renal failure.