Publications by authors named "Reynaud-Gaubert Martine"
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.
Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium.
Article Synopsis
- Circulating cell-free DNA (cfDNA) is a promising non-invasive biomarker for monitoring solid organ transplantation (SOT), with increased research showcasing its diagnostic potential, especially regarding transplant rejection.
- A systematic review of 40 studies revealed that levels of donor-derived cfDNA (dd-cfDNA) rise significantly during rejection episodes and can help distinguish between rejection and non-rejection in different transplanted organs.
- Despite its promise, cfDNA measurement still requires standardization in technology and protocols to improve diagnostic accuracy and specificity, potentially enhanced by incorporating epigenetic analyses.
Background: Systemic sclerosis-related interstitial lung disease (SSc-ILD) represents a significant cause of morbidity and mortality in Systemic Sclerosis (SSc). Mycophenolate mofetil (MMF) is currently the first line treatment for SSc-ILD. There is no recommendation on the dosage of mycophenolic acid (MPA) blood concentrations, so we aimed to study the correlation between MPA exposure and respiratory outcomes in this population.
View Article and Find Full Text PDFBackground: Circulating extracellular vesicles (EVs) have shown promising results as noninvasive biomarkers for predicting disease outcomes in solid organ transplantation. Because in situ graft cell expression of the tolerogenic molecule HLA-G is associated with acceptance after lung transplantation (LTx), we hypothesized that plasma EV-bound HLA-G (HLA-GEV) levels could predict chronic lung allograft dysfunction (CLAD) development.
Methods: We analyzed 78 LTx recipients from the Cohort-for-Lung-Transplantation cohort, all in a stable (STA) state within the first year post-LTx.
Article Synopsis
- The study aimed to explore and describe the sexual issues faced by females with cystic fibrosis (CF), an area that has been largely overlooked in previous research.
- The research involved adult females with CF who were currently or previously in sexual relationships, collecting data through a questionnaire and one-on-one interviews to gain deeper insights into their experiences.
- Results indicated that a significant majority (93.4%) experienced discomfort during intercourse, with common issues including lack of lubrication and pain, highlighting the need for increased awareness and better management of sexual health concerns among females with CF.
The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study.
Lancet Respir Med
November 2024
Background: Elexacaftor-tezacaftor-ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTR variant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor-tezacaftor-ivacaftor for people with cystic fibrosis without a F508del CFTR variant in France and to determine CFTR variant responsiveness to elexacaftor-tezacaftor-ivacaftor based on the observed clinical response.
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- Mycophenolate mofetil (MMF) is used to treat interstitial lung disease (ILD), but its pharmacokinetics in this context weren't previously known. This study aimed to describe the variability of MPA pharmacokinetics in ILD patients using population modeling.
- MPA levels were measured in 27 ILD patients over an 8-hour period after administering 1000 mg MMF twice daily, and the best fit for its pharmacokinetics was a two-compartment model influenced by factors like body weight and renal function.
- This research represents the first exploration of MPA pharmacokinetics in ILD and suggests that MPA behaves similarly to other applications, warranting further investigation.
Background: The aim of this study was to assess the impact of immunosuppression management on coronavirus disease 2019 (COVID-19) outcomes.
Methods: We performed a single-center retrospective study in a cohort of 358 lung transplant recipients (LTx) over the period from March 2020 to April 2022. All included symptomatic patients had at least one positive SARS-CoV-2 rt-PCR.
Limited data exist on the safety and effectiveness of elexacaftor-tezacaftor-ivacaftor (ETI) in people with cystic fibrosis (pwCF) and advanced lung disease. To evaluate the effects of ETI in an unselected population of pwCF and advanced lung disease. A prospective observational study, including all adults aged 18 years and older with percentage predicted forced expiratory volume in 1 second (ppFEV) ⩽ 40 who initiated ETI from December 2019 to June 2021 in France, was conducted.
View Article and Find Full Text PDFBackground: Several rare surfactant-related gene (SRG) variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing. We aimed to study the epidemiology and phenotype of lung cancer in an international cohort of SRG variant carriers.
Methods: We conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomere-related gene (TRG) variant carriers.
Introduction: Computed tomography (CT) is routinely employed on the evaluation of dyspnea, yet limited data exist on its assessment of diaphragmatic muscle. This study aimed to determine the capability of CT in identifying structural changes in the diaphragm among patients with ultrasound-confirmed diaphragmatic dysfunction.
Methods: Diaphragmatic ultrasounds conducted between 2018 and 2021 at our center in Marseille, France, were retrospectively collected.
Article Synopsis
- * The majority of patients were women, with 47.2% testing positive for certain antibodies; about 65% had another connective tissue disease, and ILD was typically diagnosed years after the SLE diagnosis.
- * Despite a generally good prognosis, with high survival rates, nearly half of the patients experienced ILD progression, and factors like cutaneous symptoms were linked to better survival outcomes.
Background And Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults.
Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed.
Background: Interstitial lung disease associated with genetic disorders of the surfactant system is a rare entity in adults that can lead to lung transplantation. Our objective was to describe the outcome of these patients after lung transplantation.
Methods: We conducted a retrospective, multicentre study, on adults who underwent lung transplantation for such disease in the French lung transplant centres network, from 1997 to 2018.
Article Synopsis
- Lung transplantation often faces challenges due to acute or chronic rejection, linked to the presence of HLA donor-specific antibodies and other autoantibodies.
- Non-classical HLA molecules, such as HLA-G, play a role in immune acceptance of lung grafts; certain isoforms are associated with poorer outcomes in transplants.
- The study aimed to investigate HLA-G antibody prevalence in lung transplant recipients, finding specific antibody reactivity rare, with non-specific responses indicating possible issues with autoantigens in the testing cells.
Background: Interstitial lung disease (ILD) and pulmonary hypertension (PH) represent the major causes of mortality in systemic sclerosis (SSc). Patients with systemic sclerosis and combined PH and ILD (SSc-PH-ILD) generally have a poor prognosis. Predictors of survival and of potential benefit of treatment are lacking in patients with SSc-PH-ILD.
View Article and Find Full Text PDFBackground And Aim: Germline mutations of telomere-related genes (TRG) induce multiorgan dysfunction, and liver-specific manifestations have not been clearly outlined. We aimed to describe TRG mutations-associated liver diseases.
Approach And Results: Retrospective multicenter analysis of liver disease (transaminases > 30 IU/L and/or abnormal liver imaging) in patients with TRG mutations.
Introduction: The diaphragm function assessed by ultrasound has been well-studied in COPD, asthma, and intensive care. However, there are only a few studies on diffuse interstitial lung disease, while dyspnea and quality of life are major issues in the management that may depend on the diaphragm.
Methods: We retrospectively included idiopathic pulmonary fibrosis (IPF) patients followed in our center (Marseille, France) between January 2020 and February 2023 who underwent diaphragmatic ultrasound.
Article Synopsis
- The study investigates how lung disease affects diaphragm structure, particularly after single-lung transplantation (SLTx), using CT scans to measure diaphragm characteristics.
- Researchers analyzed CT scans of 31 patients from Marseille, France, who had undergone SLTx between 2009 and 2022 and compared the transplanted lung's diaphragm to the native lung's.
- Results showed that after SLTx, the transplanted lung's diaphragm thickness increased significantly while its height decreased, indicating recovery potential, while there were no notable changes in the diaphragm related to the native lung.
Background: Antifibrotic agents (AFAs) are now standard-of-care for idiopathic pulmonary fibrosis (IPF). Concerns have arisen about the safety of these drugs in patients undergoing lung transplantation (LTx).
Methods: We performed a multi-centre, nationwide, retrospective, observational study of French IPF patients undergoing LTx between 2011 and 2018 to determine whether maintaining AFAs in the peri-operative period leads to increased bronchial anastomoses issues, delay in skin healing and haemorrhagic complications.
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease, predisposing to an increased risk of infection. A complete picture of these infections is lacking.
Research Question: Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.
Article Synopsis
- Chronic lung allograft dysfunction (CLAD) can manifest as bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS), and this study aimed to see if chest-CT abnormalities post-lung transplant could predict CLAD before patients experience respiratory decline.
- The study involved analyzing CT scans from 118 lung transplant patients over several years, revealing that certain CT features like moderate pulmonary artery stenosis and consolidations were associated with a higher risk of developing CLAD or even death.
- Findings suggest that routine chest CT scans after lung transplantation may help identify patients at risk for CLAD, allowing for earlier interventions.
Research Questions: Patients with severe pulmonary hypertension associated with chronic lung disease have a poor prognosis. Targeted pulmonary arterial hypertension therapies might improve exercise capacity and outcome, but there are no guidelines on treatments which are not recommended because of an unproven benefit, with discordant results from few studies in this context. The aim of our study was to evaluate targeted pulmonary arterial hypertension therapies for severe group 3 pulmonary hypertension patients.
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