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Article
Open AccessA formula for predicting emphysema extent in combined idiopathic pulmonary fibrosis and emphysema
No single pulmonary function test captures the functional effect of emphysema in idiopathic pulmonary fibrosis (IPF). Without experienced radiologists, other methods are needed to determine emphysema extent. H...
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Chapter
Overview of Lung Involvement
In systemic sclerosis (SSc), interstitial lung disease (SSc-ILD) is a major cause of morbidity and mortality in SSc with pulmonary hypertension a frequent complication. Although a rare disease in the general c...
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Article
Open AccessEffect of Nintedanib in Patients with Progressive Pulmonary Fibrosis in Subgroups with Differing Baseline Characteristics
In the INBUILD trial in patients with progressive pulmonary fibrosis other than idiopathic pulmonary fibrosis (IPF), nintedanib slowed the rate of decline in forced vital capacity (FVC; mL/year) over 52 weeks ...
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Chapter
Systemic Sclerosis and the Lung
The last decade has seen major advances in the prognostic evaluation and treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD). Risk factors for the development of SSc-ILD include diff...
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Article
Serial decline in lung volume parameters on computed tomography (CT) predicts outcome in idiopathic pulmonary fibrosis (IPF)
In patients with IPF, this study aimed (i) to examine the relationship between serial change in CT parameters of lung volume and lung function, (ii) to identify the prognostic value of serial change in CT para...
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Article
New insights into the treatment of CTD-ILD
Interstitial lung disease (ILD) can arise in a variety of connective tissue diseases (CTDs) and various treatment interventions are being explored. In 2020, advances in the treatment of CTD-associated ILD have...
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Article
Open AccessDefining genetic risk factors for scleroderma-associated interstitial lung disease
Although several genetic associations with scleroderma (SSc) are defined, very little is known on genetic susceptibility to SSc-associated interstitial lung disease (SSc-ILD). A number of common polymorphisms ...
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Article
Open AccessPsychometric properties and minimal important differences of SF-36 in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a rare disease with a median survival of 3–5 years after diagnosis with limited treatment options. The aim of this study is to assess the psychometric characteristics of ...
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Article
Open AccessIdiopathic pulmonary fibrosis: prognostic impact of histologic honeycombing in transbronchial lung cryobiopsy
Prognostic evaluation in idiopathic pulmonary fibrosis (IPF) may be important as it can guide management decisions, but the potential role of honeycomb changes in providing information about outcome and surviv...
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Article
Open AccessDiagnostic yield and risk/benefit analysis of trans-bronchial lung cryobiopsy in diffuse parenchymal lung diseases: a large cohort of 699 patients
Standardization of trans-bronchial lung cryobiopsy in diffuse parenchymal lung diseases is imminent; however, the majority of published series on cryobiopsy include a limited number of patients and are charact...
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Article
Open AccessPirfenidone improves survival in IPF: results from a real-life study
Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone’s effect on mortality and adverse events ...
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Article
Author Correction: Major lung complications of systemic sclerosis
Corrections: Table 2: the text ‘Also licensed for CTEPH; cannot be combined with a PDE5 inhibitor’ has been moved to a different row; reference for this moved statement updated.
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Article
Major lung complications of systemic sclerosis
Systemic sclerosis (SSc) is associated with high mortality owing to internal organ complications, and lung disease is the leading cause of SSc-associated death. The most notable lung complications in SSc are f...
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Article
Serial automated quantitative CT analysis in idiopathic pulmonary fibrosis: functional correlations and comparison with changes in visual CT scores
To determine whether computer-based CT quantitation of change can improve on visual change quantification of parenchymal features in IPF.
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Article
Open AccessUpregulation of citrullination pathway: From Autoimmune to Idiopathic Lung Fibrosis
Increased protein citrullination and peptidylarginine deiminases (PADIs), which catalyze the citrullination process, are central in Rheumatoid arthritis pathogenesis and probably involved in the initial steps ...
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Article
Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with i...
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Article
Open AccessRituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial
Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD in...
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Article
Open AccessChronic hypersensitivity pneumonitis: identification of key prognostic determinants using automated CT analysis
Chronic hypersensitivity pneumonitis (CHP) has a variable disease course. Computer analysis of CT features was used to identify a subset of CHP patients with an outcome similar to patients with idiopathic pulm...
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Article
Open AccessAmbulatory oxygen in fibrotic lung disease (AmbOx): study protocol for a randomised controlled trial
Fibrotic interstitial lung diseases (ILDs) are chronic and often progressive conditions resulting in substantial morbidity and mortality. Shortness of breath, a symptom often linked to oxygen desaturation on e...
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Article
Open AccessChanges in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis
Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) have been defined as events of clinically significant respiratory deterioration with an unidentifiable cause. They carry a significant mortality an...
