Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 191, Issue 3, Pages 270-274Publisher
AMER THORACIC SOC
DOI: 10.1164/rccm.201405-0856UP
Keywords
IPF; ILD; genomics; personalized medicine; immunity
Categories
Funding
- National Institutes of Health [U01HL105371, P01HL114501, U01HL108642, U01HL112707]
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The period covered by this update can be considered as the most exciting period in idiopathic pulmonary fibrosis (IPF) research. It started with the identification of genetic variants that are associated with IPF in the majority of patients and continued with discovery of molecular and genetic biomarkers that predict distinct clinical presentations of patients with IPF and potential new biological mechanisms. More importantly, the period ends with the publication of two groundbreaking studies that confirmed that two drugs, pirfenidone and nintedanib, slowed disease progression, leading to a historic approval by the FDA. In this update, we describe these key advances, their scientific and significant clinical implications, and future directions.
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