Idiopathic Pulmonary Fibrosis: Addressing the current and future
therapeutic advances along with the role of Sotatercept in the
management of pulmonary hypertension
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with
unclear etiology. Clinical manifestations include dyspnea and
nonproductive cough. Lung transplantation is the only cure, while
Pirfenidone and Nintedanib are FDA-approved drugs for slowing disease
progression. However, Saracatinib shows greater efficacy. This
literature review assesses the safety and efficacy of IPF treatments,
focusing on Pirfenidone and Nintedanib, which preserve lung function and
reduce fibrosis and inflammation. We also evaluate emerging treatments
such as saracatinib, pamrevlumab, pentraxin-2, BI 1015550, ziritaxestat,
PBI-4050, bexotegrast, BMS-986020, TD 139, dasatinib, quercetin, and
etanercept. Additional research is needed to explore the therapeutic
potential and address gaps in IPF management, including exacerbation and
associated pulmonary hypertension (PH). Immunosuppressive agents are
used to manage IPF exacerbations, while PH is a recognized comorbidity.
Clinical trials, PULSAR and SPECTRA, investigate Sotatercept as a
potential PH treatment for IPF patients, showing promising results.