Vincent Cottin

Vincent Cottin
Hospices Civils de Lyon (Centre Hospitalier Universitaire de Lyon) | CHU Lyon · National Reference center for Rare pulmonary diseases, department of respiratory medicine

MD PhD

About

1,002
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Introduction
Skills and Expertise
Additional affiliations
January 2007 - December 2011
Centre Hospitalier Universitaire de Lyon
November 1999 - present

Publications

Publications (1,002)
Article
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Multidisciplinary team (MDT) meetings, involving the integrated collaboration of healthcare professionals, are increasingly used in clinical practice to inform the diagnosis and treatment of interstitial lung diseases (ILDs). Over time, the assessment of patients with ILD has transitioned from discussions among clinicians, radiologists and patholog...
Article
Purpose of review: Idiopathic pulmonary fibrosis (IPF), characterized by relentless disease progression from the time of diagnosis, is part of a larger group of chronic fibrosing interstitial lung diseases (ILDs). A proportion of patients with non-IPF ILDs may develop, despite conventional treatment, a progressive pulmonary fibrosis (PPF), also re...
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Background: The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals: The objectives of this task force were to review the terminology,...
Article
Background Riociguat and balloon pulmonary angioplasty (BPA) are treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, randomised controlled trials comparing these treatments are lacking. We aimed to evaluate the efficacy and safety of BPA versus riociguat in patients with inoperable CTEPH. Methods In thi...
Article
Background Since the latest 2017 French guidelines, knowledge about idiopathic pulmonary fibrosis has evolved considerably. Methods Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Lung Diseases, led by the French Language Pulmonology Society (SPLF), by a coordinating group, a writing group, and a r...
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Purpose of review: Pleuroparenchymal fibroelastosis (PPFE) is a clinico-radiologic-pathologic interstitial lung disease (ILD) characterized by fibrosis that has upper lobe and subpleural predominance, involving both the visceral pleura and the subjacent subpleural lung parenchyma, and comprises dense fibroelastic changes with prominent elastosis o...
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Introduction Idiopathic pulmonary fibrosis (IPF) greatly impacts quality of life and eventually leads to premature death from respiratory failure. Inhaled treprostinil was associated with improvements in forced vital capacity (FVC) and reduced exacerbations of underlying lung disease in post hoc analyses from a phase 3 study in patients with precap...
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Objectives: To evaluate short- and long-term safety and efficacy of embolization with Onyx® for recurrent pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia (HHT). Methods: In total, 45 consecutive patients (51% women, mean (SD) age 53 (18) years) with HHT referred to a reference center for treatment of recurr...
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Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pul...
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Introduction: Rheumatoid arthritis (RA) is the most common inflammatory autoimmune disease, characterised by symmetric destructive arthritis and synovitis. Lung involvement is frequent, including in the form of interstitial lung disease (ILD). RA-ILD often presents with a radiologic and pathologic pattern of usual interstitial pneumonia, similar t...
Article
Introduction L’atteinte cardiaque est une des causes principales de décès dans la sclérodermie systémique (SSc). Ses différentes manifestations sont très hétérogènes et leurs prévalences mal connues, notamment en raison d’un manque de consensus sur leurs définitions. Nous avons voulu étudier la prévalence et l’impact pronostique des différentes att...
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Approximately 12–13% of patients with interstitial lung disease (ILD) are diagnosed with unclassifiable ILD (uILD), often despite thorough evaluation. A recent Phase 2 study (NCT03099187) described a significant effect of pirfenidone vs. placebo on forced vital capacity (FVC) measured by site spirometry in patients with progressive fibrosing uILD (...
Article
Almost 25% of patients with pulmonary fibrosis referred for lung transplantation have a germline rare variant of a telomere-related gene. Acquired TERT promoter mutations may counterbalanced the germline defect and reduce the risk of hematological complications in this population. In a series of 34 patients with a germline telomere-related gene mut...
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Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to concurrently apply their respective recommendations within a single patient, where discrimination b...
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Background: Phosphodiesterase 4 (PDE4) inhibition is associated with antiinflammatory and antifibrotic effects that may be beneficial in patients with idiopathic pulmonary fibrosis. Methods: In this phase 2, double-blind, placebo-controlled trial, we investigated the efficacy and safety of BI 1015550, an oral preferential inhibitor of the PDE4B...
Article
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Introduction: The effect of nintedanib on slowing the rate of decline in forced vital capacity (FVC) has been investigated in randomized placebo-controlled trials in subjects with idiopathic pulmonary fibrosis (IPF), other progressive fibrosing interstitial lung diseases (ILDs), and ILD associated with systemic sclerosis (SSc-ILD). We assessed the...
Article
Rationale: SARS-Cov2 infection is associated with pulmonary endothelial dysfunction. There is limited data available on the outcomes of COVID-19 in patients with pulmonary hypertension (PH), a disease characterized by pulmonary endothelial dysfunction. Objectives: To describe characteristics and outcomes of patients with precapillary PH and COVI...
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Background In the INBUILD trial in patients with progressive fibrosing interstitial lung diseases (ILDs), nintedanib reduced the rate of decline in forced vital capacity compared with placebo, with side-effects that were manageable for most patients. We used data from the INBUILD trial to characterize further the safety and tolerability of nintedan...
Article
Background: When considering the diagnosis of idiopathic pulmonary fibrosis (IPF), experienced clinicians integrate clinical features that help to differentiate IPF from other fibrosing interstitial lung diseases, thus generating a "pre-test" probability of IPF. The aim of this international working group perspective was to summarize these feature...
Article
Interstitial lung disease (ILD) secondary to drug-induced lung injury is an increasingly common cause of morbidity and mortality. The number of drugs associated with the development of ILD continues to raise, mainly due to the use of novel monoclonal antibodies and biologics for neoplastic and rheumatologic diseases, and includes, among others, che...
Article
Résumé Contexte Depuis les précédentes recommandations françaises publiées en 2017, les connaissances concernant la fibrose pulmonaire idiopathique ont évolué sur de nombreux aspects. Méthodes Des recommandations pratiques ont été établies à l’initiative du Centre coordonnateur de référence des maladies pulmonaires rares, sous l’égide de la Socié...
Article
Résumé La sclérodermie systémique (SSc) est une maladie auto-immune rare caractérisée, notamment, par la présence de manifestations fibrosantes. La pneumopathie interstitielle diffuse associée à la SSc (PID-SSc) est la première cause de mortalité liée à cette connectivite. La prise en charge de la PID-SSc a bénéficié d’avancées thérapeutiques récen...
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Background and objective: Demographic and clinical variables, measured at baseline or over time, have been associated with mortality in subjects with progressive fibrosing interstitial lung diseases (ILDs). We used data from the INPULSIS trials in subjects with idiopathic pulmonary fibrosis (IPF) and the INBUILD trial in subjects with other progre...
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Objectives: To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods: We conducted in an expert center a retrospective study betwee...
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Patients with systemic sclerosis are at high risk of developing systemic sclerosis–associated interstitial lung disease. Symptoms and outcomes of systemic sclerosis–associated interstitial lung disease range from subclinical lung involvement to respiratory failure and death. Early and accurate diagnosis of systemic sclerosis–associated interstitial...
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Background and objective Poly(A)-specific ribonuclease (PARN) mutations have been associated with familial pulmonary fibrosis. This study aims to describe the phenotype of patients with interstitial lung disease (ILD) and heterozygous PARN mutations. Methods We performed a retrospective, observational, non-interventional study of patients with an...
Article
Introduction Les pneumopathies interstitielles diffuses (PID) sont des atteintes rares du lupus érythémateux disséminé (LED) comparé à d’autres connectivites. Leurs caractéristiques sont peu décrites dans la littérature. L’objectif de l’étude était de décrire les caractéristiques cliniques, fonctionnelles, la présentation radiologique des pneumopat...
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Introduction La pneumopathie interstitielle non spécifique (PINS) a un pronostic sévère. Par analogie avec les pneumopathies interstitielles associées à la sclérodermie, la prise en charge standard de la PINS repose en première ligne sur des immunosuppresseurs tel que le mycophenolate mofetil (MMF). L’infiltration lymphocytaire CD20 pulmonaire au c...
Article
Introduction Certains patients atteints de PID en dehors de la FPI peuvent développer un phénotype fibrosant progressif (PID-FP) similaire à celui observé dans la FPI. Chez les patients atteints de FPI, il a été montré que plusieurs paramètres du TM6 pouvaient être prédictifs de la mortalité. Dans ce travail nous avons donc cherché à évaluer la val...
Article
Introduction Les évaluations fonctionnelles respiratoires de repos et d’exercice (épreuves d’effort cardio respiratoires) sont déterminantes dans la prise en charge des patients ayant une pneumopathie interstitielle. La FEPP (fibroelastose pleuro parenchymateuse) est une forme rare de pneumopathie interstitielle dont le pronostic est particulièreme...
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Introduction Les pneumopathies interstitielles diffuses (PID) associées à une mutation d’un gène codant pour une protéine du surfactant représentent une entité rare chez l’adulte pouvant nécessiter une transplantation pulmonaire (TxP). L’objectif de notre étude est de décrire l’évolution post-TxP de ces patients. Méthodes Nous avons mené une étude...
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Introduction Les rétrovirus sont une vaste famille de virus, comportant notamment des virus capables d’induire des cancers chez l’homme et l’animal. Jaagsiekte Sheep Retrovirus (JSRV) est un rétrovirus oncogène infectant les moutons et induisant un adénocarcinome pulmonaire à prédominance lépidique avec une présentation pneumonique, analogue à cert...
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Introduction Le spectre des pneumopathies infiltrantes diffuses fibrosantes progressives (PID-FP) regroupe des PID fibrosantes qui évoluent de façon similaire à la fibrose pulmonaire idiopathique (FPI) malgré un traitement conventionnel. Il est impératif d’identifier des biomarqueurs capables de dépister les patients atteints d’une PID-FP à risque...
Article
Introduction La bronchiolite chronique idiopathique (BCI) est une forme rare de bronchiolite chronique irréversible de l’adulte. L’évolution serait longue, avec un risque de progression vers une insuffisance respiratoire, mais peu d’études sont disponibles. Notre objectif était d’évaluer les caractéristiques cliniques, fonctionnelles, radiologiques...
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The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial...
Article
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Background: Real-world data regarding outcomes of idiopathic pulmonary fibrosis (IPF) are scarce, outside of registries. The claims data from the French National Health System (SNDS) were used to describe outcomes in patients diagnosed with IPF in 2015–2016 but who did not receive antifibrotic therapies. Method: Patients aged <50 years were exclude...
Article
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IntroductionThere are currently no approved treatments solely for unclassifiable interstitial lung disease (uILD); however, a recent trial showed this population can benefit from pirfenidone. We report a subgroup analysis of this trial to assess the effects of immunomodulators (concomitant mycophenolate mofetil [MMF] and/or previous corticosteroids...
Article
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The X-ray imaging field is currently undergoing a period of rapid technological innovation in diagnostic imaging equipment. An important recent development is the advent of new X-ray detectors, i.e., photon-counting detectors (PCD), which have been introduced in recent clinical prototype systems, called PCD computed tomography (PCD-CT) or photon-co...
Article
Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify predictors of survival. In a m...
Article
IntroductionPirfenidone, an antifibrotic medication for idiopathic pulmonary fibrosis (IPF), is now available in France in two formulations: tablets since April 2018, and the initial capsules form. We conducted a cohort study to describe tolerance and acceptability of capsules and/or tablets of pirfenidone in patients with IPF.Methods This study wa...
Article
Introduction Chronic pulmonary eosinophilia is a rare condition, usually highly responsive to systemic glucocorticoids, yet relapses are frequent and require long-term treatment associated with significant morbidity. Areas covered We review the main conditions causing chronic pulmonary eosinophilia and glucocorticoid-sparing agents in this setting...
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Introduction Contemporary risk assessment tools categorise patients with pulmonary arterial hypertension (PAH) as low, intermediate, or high-risk. A minority of patients achieve low-risk status with most remaining intermediate-risk. Our aim was to validate a 4-strata risk assessment approach categorising patients as low, intermediate-low, intermedi...
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There are limited epidemiologic studies describing the global burden and geographic heterogeneity of interstitial lung disease (ILD) subtypes. We found that among seventeen methodologically heterogenous studies that examined the incidence, prevalence and relative frequencies of ILDs, the incidence of ILD ranged from 1 to 31.5 per 100,000 person-yea...
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Background: Weight loss is frequently reported in patients with idiopathic pulmonary fibrosis (IPF) and may be associated with worse outcomes in these patients. Objective: The aim of this study was to investigate the relationships between body mass index (BMI) and weight loss, and outcomes over 1 year in patients with IPF. Methods: Data were i...
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Background and objective: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease that requires ongoing care and is associated with considerable socioeconomic burden. We evaluated the IPF care pathway from symptom recognition to treatment. We describe the impact of IPF on healthcare resource use (HCRU), quality of life (QoL) and work...
Article
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Background: Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in patients with idiopathic and secondary PPFE. Patients admitted to a tertiary care center (2010-2019) were included into this retros...
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Physicians are observing persisting symptoms and unexpected organ dysfunction after severe COVID‐19. In this report, we present the follow‐up of two cases of suspected interstitial lung disease following the viral infection. Up to the last month of follow‐up, both patients presented with persistent changes in imaging and pulmonary function tests. T...
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Tuberous sclerosis complex (TSC) is a rare multisystem genetic disorder affecting almost all organs with no sex predominance. TSC has an autosomal-dominant inheritance and is caused by a heterozygous mutation in either the TSC1 or TSC2 gene leading to hyperactivation of the mammalian target of rapamycin (mTOR). TSC is associated with several pulmon...
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Variable patterns of disease progression are typically observed in patients with idiopathic pulmonary fibrosis (IPF). We sought to determine the prognostic capacity of blood cell count indexes, derived from routine complete blood cell (CBC) count, in a cohort of IPF patients. The neutrophil-to-lymphocyte ratio (NLR), derived neutrophil-to-lymphocyt...
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Background: Epilepsy is the most common neurological manifestation in individuals with tuberous sclerosis complex (TSC). However, real-world evidence on diagnosis and treatment patterns is limited. Here, we present data from TuberOus Sclerosis registry to increase disease Awareness (TOSCA) on changes in patterns of epilepsy diagnosis, treatments, a...