CHEST Guidelines-Therapeutic-Approach-to-Adult-Fibrotic-Lung-Diseas

Therapeutic-Approach-to-Adult-Fibrotic-Lung-Diseas

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The article "Therapeutic Approach to Adult Fibrotic Lung Diseases" by Ayodeji Adegunsoye and Mary E. Strek discusses the challenges and advancements in treating fibrotic interstitial lung diseases (ILDs) such as idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis (cHP), and connective tissue disease-related ILD (CTD-ILD). These conditions are highly fatal, primarily due to respiratory failure and related comorbidities.<br /><br />Key treatments for IPF include newly approved antifibrotic agents nintedanib and pirfenidone, which have been shown to slow lung function decline in clinical trials. There is uncertainty regarding the use of these medications for cHP and CTD-ILD without rigorous clinical trials proving safety and efficacy. Historically, anti-inflammatory and immunosuppressive therapies have been used, but evidence supporting their benefit is minimal.<br /><br />Pirfenidone, an oral medication, inhibits critical pathways involved in fibrosis, showing success in reducing the decline in lung function among IPF patients. Multiple trials, including ASCEND and CAPACITY, demonstrated improved outcomes such as slower FVC decline. Nintedanib, acting on multiple growth factor receptors, also displays efficacy in slowing disease progression, supported by TOMORROW and INPULSIS trials.<br /><br />Furthermore, attention to managing associated comorbid conditions like pulmonary hypertension, gastroesophageal reflux, and sleep apnea is crucial to enhancing life quality and survival. Early evaluation for lung transplantation is advised for those with IPF and progressive fibrotic ILD.<br /><br />The article also explores potential future therapies, including stem cell treatments and combination therapies to improve treatment efficacy. While new therapies show promise, ongoing research and clinical trials are essential for advancing treatment strategies and improving patient outcomes in fibrotic lung diseases.

Keywords

fibrotic lung diseases

idiopathic pulmonary fibrosis

chronic hypersensitivity pneumonitis

connective tissue disease-related ILD

antifibrotic agents

nintedanib

pirfenidone

lung transplantation

clinical trials

stem cell treatments