Microscopic analysis of biomarkers in pulmonary fibrotic tissue, key in the development of innovative drugs for Idiopathic Pulmonary Fibrosis by Ferrer.

Diseases

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a disease in which lung tissue is damaged and scarring occurs that progressively replaces healthy tissue. IPF is characterized by a progressive loss of the lungs' ability to transfer oxygen to the blood and is the most common of the idiopathic interstitial pneumonias. IPF rarely appears before the age of 40 and is estimated to affect 17.7 people per 100,000 worldwide¹.

Top view of a person placing a blue and white digital pulse oximeter on another person's index finger on a white surface.

Its cause is unknown (idiopathic) although it may be associated with smoking and certain genetic dispositions. In addition, gastroesophageal reflux (acid reflux or heartburn), certain viral infections, air pollution, and some occupational exposures can be risk factors for IPF.

This disease can present with a wide range of symptoms, including shortness of breath with or without activity, persistent dry cough, and fatigue, ultimately resulting in respiratory failure and death. One of the reasons why IPF can be diagnosed late or misdiagnosed is that the symptoms can be similar to those of other lung diseases.

One pair of hands holds and closely examines a piece of paper with the results of an electrocardiogram printed out.

Close-up of two people affectionately holding the hand of a patient wearing an ID bracelet on a hospital bed.

IPF is a serious disease and has a significant impact on patients' quality of life. Its burden includes high medical costs, dependency on care, and limited survival after diagnosis (with a median survival of 2 to 5 years if left untreated²).

Patients with IPF experience disease progression at different rates: some progress slowly and live with pulmonary fibrosis for many years, while others worsen more quickly. However, advances are being made in research that allow people to live longer and enjoy a better quality of life.

Maher TM, Bendstrup E, Dron L, Langley J, Smith G, Khalid JM, et al. Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021 Jul 7; 22(1):197.
Molina-Molina M, Aburto M, Acosta O, Ancochea J, Rodríguez-Portal JA, Sauleda J, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Expert Rev Respir Med. 2018 Jul; 12(7):537-539.
Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022 May 1; 205(9):E18-E47.