Vincent Cottin
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View article: Efficacy and safety of DANAZOL for pulmonary fibrosis or bone-marrow failure associated with telomere-related gene mutation
Efficacy and safety of DANAZOL for pulmonary fibrosis or bone-marrow failure associated with telomere-related gene mutation Open
Telomere-related gene (TRG) pathogenic variants are detected in ∼30% of familial pulmonary fibrosis (PF) cases. Danazol, a synthetic sex hormone with androgenic properties, has been found associated with telomere elongation and hematologic…
View article: Molecular Investigation in Early‐Onset Interstitial Lung Disease: Results From 699 Unrelated Patients
Molecular Investigation in Early‐Onset Interstitial Lung Disease: Results From 699 Unrelated Patients Open
Background and Objective Interstitial lung diseases ( ILDs ) are rare and severe respiratory conditions that may ultimately result in pulmonary fibrosis ( PF ). The objective of this study was to present the results of molecular diagnosis …
View article: Uncommon Cause of Pulmonary Hypertension: Imaging Diagnosis of Cardiac Myxoma Embolism
Uncommon Cause of Pulmonary Hypertension: Imaging Diagnosis of Cardiac Myxoma Embolism Open
We report an original case of pulmonary hypertension with artery aneurysms due to the cell proliferation of a right atrial myxoma with multi-modality imaging. Only three cases have been reported in the literature. The description of endolu…
View article: Clinical Characteristics of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)
Clinical Characteristics of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Open
RATIONALE DIPNECH is characterized by diffuse bronchial hyperplasia of pulmonary neuroendocrine cells which are situated within the walls of bronchi and bronchioles. Presenting symptoms are non-specific and the clinical course varies, maki…
View article: The phenotypic state of biallelic <i>SERPINA1 Q0</i> variants in α <sub>1</sub> -antitrypsin deficiency
The phenotypic state of biallelic <i>SERPINA1 Q0</i> variants in α <sub>1</sub> -antitrypsin deficiency Open
Biallelic SERPINA1 Q0 variants lead to early-onset emphysema even in nonsmokers, mandating early-life preventive and therapeutic measures. ZZ patients' lungs on shut-off transcription strategies equating them to "null" variants should also…
View article: Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis
Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis Open
Background and Objective Approximately 30% of individuals diagnosed with familial pulmonary fibrosis (FPF) exhibit a pathogenic variant upon genetic analysis. We established a genetic Multidisciplinary Discussion (geneMDD) aimed to enhance…
View article: Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients
Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients Open
AI-based automatic quantification of lesions from chest-CT images in SSc-ILD is correlated with physiological parameters and can help in disease evaluation. Further clinical multicentric validation is necessary in order to confirm its pote…
View article: Characteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data
Characteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data Open
LAM is a rare multi-cystic lung disease for which treatment with sirolimus is indicated in cases of moderate or severe lung disease or declining lung function. The aim of this study was to describe patients treated with sirolimus for LAM a…
View article: Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort
Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort Open
Background Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample‐size and/or follow‐up duration are available. Methods The COHESion study is a national p…
View article: Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients
Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients Open
Background: Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional, and imaging characteristics of interstitial lung disease in pa…
View article: Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study
Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study Open
Background We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process. Methods Foll…
View article: Childhood interstitial lung disease survivors in adulthood: a European collaborative study
Childhood interstitial lung disease survivors in adulthood: a European collaborative study Open
Background Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers a…
View article: Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients
Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients Open
Background. Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional and imaging characteristics of interstitial lung disease in pat…
View article: Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis
Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis Open
Background and aims Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart inv…
View article: Efficacy and Safety of Admilparant, an LPA<sub>1</sub> Antagonist, in Pulmonary Fibrosis: A Phase 2 Randomized Clinical Trial
Efficacy and Safety of Admilparant, an LPA<sub>1</sub> Antagonist, in Pulmonary Fibrosis: A Phase 2 Randomized Clinical Trial Open
Rationale: Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) have high morbidity and mortality; thus, novel treatments are needed. Objectives: Assess efficacy and safety of admilparant (BMS-986278), an oral lysop…
View article: The interstitial lung disease patient pathway: from referral to diagnosis
The interstitial lung disease patient pathway: from referral to diagnosis Open
Background Suspected interstitial lung disease (ILD) patients may be referred to an ILD-specialist centre or a non-ILD-specialist centre for diagnosis and treatment. Early referral and management of patients at ILD-specialist centres has b…
View article: Pulmonary hypertension associated with lung diseases
Pulmonary hypertension associated with lung diseases Open
Pulmonary hypertension (PH) associated with chronic lung disease (CLD) is both common and underrecognised. The presence of PH in the setting of lung disease has been consistently shown to be associated with worse outcomes. Recent epidemiol…
View article: Effects of nintedanib on circulating biomarkers of idiopathic pulmonary fibrosis
Effects of nintedanib on circulating biomarkers of idiopathic pulmonary fibrosis Open
Background Biomarkers that change in response to nintedanib in subjects with idiopathic pulmonary fibrosis (IPF) would be valuable. We investigated the effects of nintedanib on circulating biomarkers in subjects with IPF in the INMARK tria…