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 …

This expert group consensus statement emphasises the need for standardising the definition
of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis …

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ
involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous …

Background Systemic sclerosis is a connective tissue disease characterised by multiorgan
fibrosis with an autoimmune background and poor prognosis. Although a few drugs have …

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 Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis
and a leading cause of systemic sclerosis–related death. Nintedanib, a tyrosine kinase …

Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by
immune dysregulation and progressive fibrosis that typically affects the skin, with variable …

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