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.