Keywords
Idiopathic Pulmonary Fibrosis; Usual Interstitial Pneumonia; Interstitial Lung diseases
Abstract
Idiopathic Pulmonary Fibrosis (IPF) is a progressive and often fatal disease that usually manifests with dyspnea, cough and/or fatigue. The diagnosis of IPF requires the exclusion of known causes of pulmonary f ibrosis. The diagnosis and management of patients with IPF ideally should be done in specialized interstitial lung disease centers in order to provide patients with the best possible care; this includes pharmacological and non-pharmacological therapeutic options to slow down the progression of the disease, to manage symptoms and to improve the quality of life of patients with IPF. The main treatment options include antifibrotic drugs, opioids, supplementary oxygen, pulmonary rehabilitation and lung transplantation. This review summarizes the guidelines and scientific evidence that support the various therapeutic options currently used in the management of patients with IPF. We also summarize the evidence on the therapeutic options for pulmonary hypertension and acute exacerbations of IPF, two complications associated with increased risk of death in IPF patients.
Citation
Alyami S, Yang R and Moran-Mendoza O. Idiopathic Pulmonary Fibrosis Management Review. SM J Biomed Eng. 2017; 3(2): 1018.