Immune microenvironment in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary renal disorder characterized by the progressive development of fluid-filled cysts within the kidneys, leading to renal dysfunction and potentially life-threatening complications. While ADPKD has long been considered a primarily genetic disorder, emerging evidence suggests that the immune microenvironment within the kidney plays a pivotal role in disease progression and severity. This review explored the intricate interplay between immune cells, inflammatory microenvironment, inflammatory pathways, complement system, and ADPKD, shedding light on the various immune components and mechanisms contributing to ADPKD pathogenesis. Key findings suggest that renal immune cell infiltration, inflammation, and the complement system could take part in cyst growth, renal fibrosis, and ADPKD progression. Inflammation, in particular, stands out as a prime candidate for therapeutic intervention. Moreover, recent studies have unveiled the involvement of immune checkpoints, such as PD-1 and its ligand PD-L1, in modulating the immune response within ADPKD kidneys. In conclusion, this review highlights the emerging paradigm shift in the understanding of ADPKD, emphasizing the pivotal role of the immune microenvironment in disease pathogenesis. Targeted therapies aimed at modulating immune responses and addressing immune-related checkpoints may hold promise for the development of novel treatments to improve the clinical outcomes of ADPKD patients.