Long-term Course and Prognosis of Idiopathic Pulmonary Fibrosis in the New Millennium

A Novel Genomic Signature with Translational Significance for Human Idiopathic Pulmonary Fibrosis

“…In addition, the translational gene set was also able to ORIGINAL RESEARCH segregate control subjects from patients with IPF in two publicly available, independent clinical IPF/control datasets (GSE10667 and GSE24206), thereby supporting the findings ( Figure E17). Furthermore, in patients with IPF, the expression levels of the translational gene markers, GREM1, MMP7, CTHRC1, and FHL2, showed a significant negative correlation with percent diffusing capacity of carbon monoxide (%DL CO ) (GREM1, r = 20.68, adjusted P = 4.2e-25; MMP7, r = 20.64, adjusted P = 3.4e-22; CTHRC1, r = 20.64, adjusted P = 3.4e-22; and FHL2, r = 20.61, adjusted P = 1.7e-19), a parameter associated with disease severity (34,35). IPF samples obtained from lung transplantation (category 1, lung explant single; and category 2, lung explant bilateral) showed higher MMP7 expression and a lower %DL CO compared with IPF samples obtained from lung biopsies (category 3, lung lobectomy; or category 4, lung biopsy) ( Figure 5).…”

Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis: Experience from 92 Sites in an Open-Label US Expanded Access Program

“…Idiopathic pulmonary fibrosis (IPF) is a progressive, unpredictable, and irreversible fibrosing lung disease with median survival rates estimated at 2-5 years from the time of diagnosis [1][2][3][4]. Pirfenidone is an oral antifibrotic agent whose clinical efficacy and safety have been demonstrated in three multinational, randomized controlled phase 3 trials [5,6].…”

Stem cell therapy for idiopathic pulmonary fibrosis: How far are we from the bench to the bedside?

“…Patients with late-stage IPF mostly die for respiratory failure, accompanying cardiac diseases or lung infections. Whereas the incidence of IPF seems to be low (approximately 2 to 29 cases per 100, 000 individuals), the 5-year survival rate of confirmed IPF patients who receive lung transplantation or not has never exceeded 50%, which is even lower than many malignancies [1,2].…”