Patients with idiopathic pulmonary fibrosis (IPF) showed improved lung function and reversal of lung fibrosis after 12 weeks of treatment with an investigational inhibitor of the Hedgehog signaling pathway. Data from a phase 2a trial with the novel oral agent ENV-101 indicated improvements in forced vital capacity (FVC) and lung function, potentially offering a disease-modifying therapy for IPF. The activation of the Hedgehog pathway in response to repetitive epithelial injury causes fibrosis in IPF, and ENV-101 blocks this pathway, leading to apoptosis of myofibroblasts involved in fibrotic matrix formation. The study showed promising results, with plans for further trials in patients with IPF.
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Hedgehog Pathway Inhibitor Improves IPF
