vismodegib + pirfenidone

**vismodegib + pirfenidone** is an investigational oral combination therapy evaluated for idiopathic pulmonary fibrosis (IPF). Vismodegib is a small-molecule inhibitor of the hedgehog signaling pathway, specifically targeting the Smoothened (SMO) protein, believed to be upregulated in IPF. Pirfenidone is an approved small-molecule antifibrotic agent that inhibits transforming growth factor beta (TGF-β) and tumor necrosis factor alpha (TNF-α), reducing fibroblast proliferation and collagen production. The combination was studied in a phase 1b open-label trial to assess safety and tolerability, showing adverse events consistent with individual profiles of both drugs, most notably muscle spasms associated with vismodegib. The research did not identify new safety signals, but tolerability issues limited further development for IPF. The trial was sponsored by F. Hoffmann-La Roche and Genentech[1][2].

Brand names

ErivedgeEsbriet

Other names

Vismodegib and Pirfenidone combination