Pharmacological treatment of pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a condition that has been described as alveolar collapse and thickening, which correlate with dysregulated surfactant production and injury to type 2 alveolar cells. As resolution of chest computed tomography has improved, especially with the development of high-resolution computed tomography (HRCT), the diagnostic measures adopted for pulmonary fibrosis has gradually shifted from biopsy to HRCT. This shift towards HRCT has aided in diagnostic evaluation and detection of the therapeutic and adverse effects of drugs for pulmonary fibrosis. Further, after the endpoint was changed to forced vital capacity, significant improvements are being observed in clinical trial outcomes. Currently active clinical trials are replacing lung biopsy with HRCT. In 2014, pirfenidone and nintedanib gained approval for tandem use in patients with IPF. These drugs were found to not only reduce the progression of pulmonary fibrosis, but also the acute exacerbation and mortality associated with the condition. These drugs showed consistent benefits regardless of the severity of patients' symptoms. Additionally, both nintedanib and pirfenidone were found to be effective in patients with advanced pulmonary fibrosis that was not classified as IPF. Nintedanib has been shown to reduce forced vital capacity in interstitial lung diseases associated with systemic sclerosis. In the next three to five years, many changes in treatment are expected, not only for IPF, but also for the entire spectrum of pulmonary fibrotic diseases. Pirfenidone and nintedanib are now considered standard treatments for IPF and few other fibrotic lung diseases. Clinicians treating patients with pulmonary fibrosis should keep themselves updated with the results of clinical trials that are currently underway.