Pirfenidone treatment attenuates fibrosis in autosomal dominant polycystic kidney disease.

ABSTRACT:

Introduction

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the formation of fluid filled cysts, progressive fibrosis and chronic inflammation, often leading to kidney failure. Renal fibrosis in ADPKD is primarily driven by myofibroblast activation and excessive extracellular matrix (ECM) accumulation, which contribute to disease progression. Here we investigated the therapeutic potential of pirfenidone, an antifibrotic drug, on myofibroblast activity, ECM production, and ADPKD progression.

Methods

Primary cultures of myofibroblasts from human ADPKD kidneys were treated with pirfenidone in vitro, and cell proliferation, migration, contractility and changes in ECM production were measured. In vivo, the effect of pirfenidone on cyst grow

SUBMITTER: Remadevi V 

PROVIDER: S-EPMC12407920 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature