An understanding of the mechanisms underlying pulmonary fibrosis remains elusive. Once
believed to result primarily from chronic inflammation, it is now clear that inflammation and …

Rationale: The 2002 American Thoracic Society/European Respiratory Society classification
of idiopathic interstitial pneumonias identified nonspecific interstitial pneumonia (NSIP) as a …

… Dr du Bois has served as a consultant, study steering committee member, or chairman of
a steering committee for Actelion, Boehringer-Ingelheim, Bayer, Genzyme, InterMune, and …

Background Nintedanib (formerly known as BIBF 1120) is an intracellular inhibitor that
targets multiple tyrosine kinases. A phase 2 trial suggested that treatment with 150 mg of …

Background Idiopathic pulmonary fibrosis is a progressive and fatal lung disease with inevitable
loss of lung function. The CAPACITY programme (studies 004 and 006) was designed …

Background Idiopathic pulmonary fibrosis is a progressive lung disease with a high mortality
rate. Because the signaling pathways activated by several tyrosine kinase receptors have …

Rationale: In interstitial lung disease complicating systemic sclerosis (SSc-ILD), the optimal
prognostic use of baseline pulmonary function tests (PFTs) and high-resolution computed …

Background The mutations that have been implicated in pulmonary fibrosis account for only
a small proportion of the population risk. Methods Using a genomewide linkage scan, we …

The hallmark of idiopathic pulmonary fibrosis (IPF) is the myofibroblast, the cellular origin of
which in the lung is unknown. We hypothesized that alveolar epithelial cells (AECs) may …

Background: The clinical utility of bronchoalveolar lavage fluid (BAL) cell analysis for the
diagnosis and management of patients with interstitial lung disease (ILD) has been a subject of …