Pathogenic mechanisms underlying idiopathic pulmonary fibrosis

BJ Moss, SW Ryter, IO Rosas - Annual Review of Pathology …, 2022 - annualreviews.org
The pathogenesis of idiopathic pulmonary fibrosis (IPF) involves a complex interplay of cell
types and signaling pathways. Recurrent alveolar epithelial cell (AEC) injury may occur in …

Immune dysregulation as a driver of idiopathic pulmonary fibrosis

K Shenderov, SL Collins, JD Powell… - The Journal of clinical …, 2021 - Am Soc Clin Investig
Idiopathic pulmonary fibrosis (IPF) affects hundreds of thousands of people worldwide,
reducing their quality of life and leading to death from respiratory failure within years of …

Mechanisms of fibrosis: therapeutic translation for fibrotic disease

TA Wynn, TR Ramalingam - Nature medicine, 2012 - nature.com
Fibrosis is a pathological feature of most chronic inflammatory diseases. Fibrosis, or
scarring, is defined by the accumulation of excess extracellular matrix components. If highly …

An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management

G Raghu, HR Collard, JJ Egan, FJ Martinez… - American journal of …, 2011 - atsjournals.org
This document is an international evidence-based guideline on the diagnosis and
management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American …

The role of immune and inflammatory cells in idiopathic pulmonary fibrosis

O Desai, J Winkler, M Minasyan, EL Herzog - Frontiers in medicine, 2018 - frontiersin.org
The contribution of the immune system to idiopathic pulmonary fibrosis (IPF) remains poorly
understood. While most sources agree that IPF does not result from a primary …

Integrating mechanisms of pulmonary fibrosis

TA Wynn - Journal of Experimental Medicine, 2011 - rupress.org
Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited
therapeutic options. Although research on the pathogenesis of pulmonary fibrosis has …

Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences

M Natsuizaka, H Chiba, K Kuronuma… - American journal of …, 2014 - atsjournals.org
Rationale: Idiopathic pulmonary fibrosis (IPF) has an unknown etiology and poor prognosis.
Several large-scale epidemiologic studies have been conducted predominantly in Western …

Clinical course and prediction of survival in idiopathic pulmonary fibrosis

B Ley, HR Collard, TE King Jr - American journal of respiratory and …, 2011 - atsjournals.org
Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease
of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some …

Immune mechanisms in pulmonary fibrosis

S Kolahian, IE Fernandez, O Eickelberg… - American journal of …, 2016 - atsjournals.org
Pulmonary fibrosis, particularly idiopathic pulmonary fibrosis, represents a chronic and
progressive disease with high mortality and limited therapeutic options. Excessive …

Host responses in tissue repair and fibrosis

JS Duffield, M Lupher, VJ Thannickal… - Annual Review of …, 2013 - annualreviews.org
Myofibroblasts accumulate in the spaces between organ structures and produce
extracellular matrix (ECM) proteins, including collagen I. They are the primary “effector” cells …