Pirfenidone is an orally active small molecule that has recently been evaluated in large
clinical trials for the treatment of idiopathic pulmonary fibrosis, a fatal disease in which the …

Lysosome-related organelles (LROs) are a heterogeneous group of vesicles that share
various features with lysosomes, but are distinct in function, morphology, and composition …

Idiopathic pulmonary fibrosis (IPF) is a fatal disorder without an effective therapy to date. In a
double-blind, randomized, placebo-controlled trial, 107 patients were prospectively …

Background Early detection and treatment for interstitial lung disease (ILD) in patients with
rheumatoid arthritis (RA) may ameliorate disease progression. The objective of this study …

Pirfenidone is an oral antifibrotic agent that benefits diabetic nephropathy in animal models,
but whether it is effective for human diabetic nephropathy is unknown. We conducted a …

Background Pirfenidone (PFD) is effective for pulmonary fibrosis (PF), but its action
mechanism has not been fully explained. This study explored the signaling pathways …

Intestinal fibrosis is considered an inevitable complication of Crohn's disease (CD) that
results in symptoms of obstruction and stricture formation. Endoscopic or surgical treatment …

1. INTRODUCTION 1.1 An overview of the ILD guideline Since the publication of the first
BTS guidelines for diffuse lung disease nearly 10years ago, 1 the specialty has seen …

Background Idiopathic pulmonary fibrosis (IPF) is characterized by the insidious onset of
dyspnea or cough. However, a subset of patients has a short duration of symptoms with …

Hermansky–Pudlak syndrome (HPS) is a group of 10 autosomal recessive multisystem
disorders, each defined by the deficiency of a specific gene. HPS‐associated genes encode …