General approach to Idiopathic Pulmonary Fibrosis

Abstract

Objective: To analyze the characteristics of Idiopathic Pulmonary Fibrosis (IPF). Bibliographic review: Idiopathic pulmonary fibrosis is a chronic and progressive interstitial lung disease (ILD) characterized by fibrosis, inflammation and destruction of lung architecture. Damage to the alveolar epithelium and abnormal wound repair are considered key factors in the development of this disease. Today in the world, three million people suffer from this disease and a meta-analysis, published in 2015, predicts that this number will double by 2030. The causes of IPF remain unknown, although it is thought to result from a combination of genetic and environmental factors. Final considerations: IPF consists of a fibrotic and progressive lung disease, whose life expectancy after diagnosis is 3 to 5 years. Thus, the treatment of this disease should be done quickly with the available antifibrotic therapy, Pirfenidone and Nintedanib, in order to also manage the side effects of these drugs. Therapy with these drugs, despite not delaying the progression of the disease, is able to reduce exacerbations of the disease, thus reducing mortality and improving the quality of life of patients.