Novel insights into the pathogenesis, evaluation, and treatment of interstitial lung disease

TOPICAL COLLECTIONS

Guest Editors

Traci Adams

University of Texas Southwestern Medical Center

Dr. Traci Adams is an Assistant Professor of Medicine and Director of the Interstitial Lung Disease program at the University of Texas Southwestern Medical Center in Dallas, Texas. She completed internal medicine residency training at Massachusetts General Hospital and pulmonary and critical care fellowship at the University of Texas Southwestern Medical Center. Her research interests include hypersensitivity pneumonitis and the use of bronchoscopic techniques to evaluate interstitial lung disease.

Claudio Tirelli

ASST Santi Paolo e Carlo, Milan, Italy, Respiratory Unit

Dr. Claudio Tirelli is a Pulmonologist. His research is mainly dedicated to rare pulmonary diseases, especially Interstitial Lung Diseases (ILD), focusing on Idiopathic Pulmonary Fibrosis and Connective Tissue Disease related to ILD. Clinical and biological aspects of these diseases are themes of increasing investigation. Novel approaches to ILD and experimental methods for drug development represent a global challenge, which will be encouraged in this Methods Collection.

Collection Overview

Interstitial lung disease (ILD) is characterized by inflammation and/or fibrosis of the lung parenchyma. ILD is an umbrella term that encompasses > 100 distinct conditions. The subtype of ILD can impact treatment and prognosis. Idiopathic pulmonary fibrosis (IPF), a subtype of ILD, is radiographically and histologically associated with the usual interstitial pneumonia (UIP) pattern. In the absence of antifibrotic therapy, IPF may lead to death within 3-5 years of diagnosis, although lung transplantation remains the only curative treatment. Fibroblasts are key cellular elements in the pathogenesis of IPF, characterized by the progressive replacement of lung tissue with fibrosis. Other subtypes of ILD may share a similar mechanism.

Precise diagnosis of the subtype of ILD is essential for treatment. Historically, surgical lung biopsy (SLB) was used routinely to determine ILD subtype; however, this procedure carries a mortality of 1-2%. Therefore, advances in recent years have included the development of noninvasive and minimally invasive approaches to the diagnostic evaluation of patients with ILD. These advances include the measurement of leukocyte telomere length, genomic classifiers, cryobiopsy, and advanced imaging techniques, and many of these techniques have been incorporated into recent guidelines. However, widespread use of these techniques has been limited by local expertise outside of established ILD centers.

The current JoVE collection may include (but is not limited to) the following research areas:

Advanced research techniques relative to IPF pathogenesis
Molecular and cellular biology of the epithelial-mesenchymal transition
Genetic and epigenetic markers of susceptibility to fibrotic ILD
Pharmacological response to antifibrotics, immunological and surgical advances in lung transplantation for ILD
Animal models of ILD
Standard and innovative approaches in the diagnosis of ILD with a particular emphasis on noninvasive and minimally invasive techniques