In patients with diffuse cutaneous systemic sclerosis, developing progressive skin fibrosis within 1 year is associated with a decline in lung function and worse survival.
Patients with idiopathic pulmonary fibrosis with better CT indices such as FEV1 and mean lung attenuation had improved outcomes.
Researchers retrospectively analyzed the association among physiologic measurements, survival, and quantitative high-resolution computed tomographic indices in patients with idiopathic pulmonary fibrosis.
Patients with interstitial lung disease who had honeycombing on chest computed tomography had higher mortality rates than patients without honeycombing.
Fewer than 25% of patients with idiopathic pulmonary fibrosis who were treated with the antifibrotic drug pirfenidone showed signs of disease progression.
Lung‐epithelial‐derived surfactant protein has been identified as a biomarker of systemic sclerosis-interstitial lung disease.
A new clinical practice guidelines summary for clinicians regarding idiopathic pulmonary fibrosis has been released, which describes clinical manifestations and diagnostic interventions for the disease.
Oral PBI-4050 alone or in combination with nintedanib or pirfenidone was well tolerated and associated with no serious adverse events in patients with idiopathic pulmonary fibrosis.