Over 200 interstitial lung diseases, from ultra rare to relatively common, are recognised.
Most interstitial lung diseases are characterised by inflammation or fibrosis within the …

Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune
connective-tissue disease. Once considered an untreatable and unpredictable condition …

Background Pirfenidone has been shown to slow disease progression in patients with
idiopathic pulmonary fibrosis (IPF). However, there are few treatment options for progressive …

Background A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in
systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial …

The term interstitial lung abnormalities refers to specific CT findings that are potentially
compatible with interstitial lung disease in patients without clinical suspicion of the disease …

Background The INBUILD trial investigated the efficacy and safety of nintedanib versus
placebo in patients with progressive fibrosing interstitial lung diseases (ILDs) other than …

Background At present, no approved pharmacotherapies are available for unclassifiable
interstitial lung disease (ILD), which is characterised by progressive fibrosis of the lung. We …

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who
have inexorable progression of pulmonary fibrosis despite treatment, which is known as the …

Background Preclinical data have suggested that nintedanib, an intracellular inhibitor of
tyrosine kinases, inhibits processes involved in the progression of lung fibrosis. Although the …

Objectives To identify overall disease course, progression patterns and risk factors
predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis …