Conclusion
Recent understanding of Idiopathic Pulmonary Fibrosis (IPF) reveals it
as a progressive disease of the lung interstitium, characterized by
fibrotic alveolar remodeling and irreversible loss of pulmonary
function. While previous management was symptomatic, recent clinical
trials have focused on developing effective treatments. Current
modalities include antifibrotic agents (nintedanib and pirfenidone) and
promising options like Saracatinib, pamrevlumab, and rhPTX-2.
Investigational therapies such as BI 1015550, ziritaxestat, PBI-4050,
PLN 74809, BMS-986020, and TD139 require further evaluation. The
combination of dasatinib and quercetin shows potential as a senolytic
therapy. Ongoing research is crucial for the assessment of the efficacy,
safety, and use of these treatments in the management of IPF.
Sotatercept has demonstrated positive outcomes in preclinical models and
trials for treating PH in IPF patients, reversing remodeling and
reducing pulmonary vascular resistance. The SPECTRA trial supports its
effectiveness, but more studies are required for the evaluation of its
efficacy and safety. Managing IPF exacerbations is challenging due to
limited guidelines. High steroid doses and pulse methylprednisolone lack
strong evidence and may harm AE-IPF cases. Retrospective studies suggest
early steroid reduction for better outcomes, though current guidelines
still recommend their use. Conversely, immunosuppressive medication like
cyclophosphamide has shown unfavorable outcomes and increased mortality
rates in trials. Advancements in IPF treatment hold promise for
improving patient outcomes, but ongoing research and trials are
essential to fully evaluate the safety, efficacy, and practical
application of those treatments in IPF management.