Restrictive Lung Disease

Progressive Skin Fibrosis in SSc Associated With Lung Function Decline

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.

Idiopathic Pulmonary Fibrosis Prognosis Predicted by CT Parameters

Patients with idiopathic pulmonary fibrosis with better CT indices such as FEV1 and mean lung attenuation had improved outcomes.

Lung CT Helps Predict Severity, Mortality in Idiopathic Pulmonary Fibrosis

Researchers retrospectively analyzed the association among physiologic measurements, survival, and quantitative high-resolution computed tomographic indices in patients with idiopathic pulmonary fibrosis.

CT Honeycombing in Interstitial Lung Disease Linked to Higher Mortality Rates

Patients with interstitial lung disease who had honeycombing on chest computed tomography had higher mortality rates than patients without honeycombing.

Pirfenidone Slows Long-Term Progression of Idiopathic Pulmonary Fibrosis

Fewer than 25% of patients with idiopathic pulmonary fibrosis who were treated with the antifibrotic drug pirfenidone showed signs of disease progression.

Biomarkers Predictive of Systemic Sclerosis-Interstitial Lung Disease Identified

Lung‐epithelial‐derived surfactant protein has been identified as a biomarker of systemic sclerosis-interstitial lung disease.

Summary of 2018 Idiopathic Pulmonary Fibrosis Diagnostic Guidelines

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.

New Idiopathic Pulmonary Fibrosis Treatment Safe, Likely Effective

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.