Article
Rheumatology
Cosimo Bruni, Mariaelena Occhipinti, Michael Pienn, Gianna Camiciottoli, Maurizio Bartolucci, Silvia Laura Bosello, Christian Payer, Zoltan Balint, Anna Rita Larici, Alessandra Tottoli, Lorenzo Tofani, Enrico De Lorenzis, Gemma Lepri, Silvia Bellando-Randone, Amelia Spinella, Dilia Giuggioli, Francesco Masini, Giovanna Cuomo, Federico Lavorini, Stefano Colagrande, Horst Olschewski, Marco Matucci-Cerinic
Summary: Recent advancements have made it possible to quantitatively assess lung vascular and parenchymal changes in thoracic CT images using automated software tools. In this study, the researchers investigated the vessel parameters of patients with SSc and correlated them with interstitial lung disease (ILD) features. The results showed that the CT vessel parameters increased in parallel with ILD extent and functional impairment, suggesting that they may serve as a biomarker of SSc-ILD severity.
Article
Medicine, Research & Experimental
Erin M. Wilfong, Katherine N. Vowell, Kaitlyn E. Bunn, Elise Rizzi, Narender Annapureddy, Rosemarie B. Dudenhofer, April Barnado, Rachel H. Bonami, Joyce E. Johnson, Leslie J. Crofford, Peggy L. Kendall
Summary: This study found that CD21(lo/neg) cells are significantly increased in systemic sclerosis patients with interstitial lung disease, indicating a potential biomarker for this condition.
CLINICAL AND EXPERIMENTAL MEDICINE
(2022)
Article
Rheumatology
David Roofeh, Kevin K. Brown, Ella A. Kazerooni, Donald Tashkin, Shervin Assassi, Fernando Martinez, Athol U. Wells, Ganesh Raghu, Christopher P. Denton, Lorinda Chung, Anna-Maria Hoffmann-Vold, Oliver Distler, Kerri A. Johannson, Yannick Allanore, Eric L. Matteson, Leticia Kawano-Dourado, John D. Pauling, James R. Seibold, Elizabeth R. Volkmann, Simon L. F. Walsh, Chester Oddis, Eric S. White, Shaney L. Barratt, Elana J. Bernstein, Robyn T. Domsic, Paul F. Dellaripa, Richard Conway, Ivan Rosas, Nitin Bhatt, Vivien Hsu, Francesca Ingegnoli, Bashar Kahaleh, Puneet Garcha, Nishant Gupta, Surabhi Khanna, Peter Korsten, Celia Lin, Stephen C. Mathai, Vibeke Strand, Tracy J. Doyle, Virginia Steen, Donald F. Zoz, Juan Ovalles-Bonilla, Ignasi Rodriguez-Pinto, Padmanabha D. Shenoy, Andrew Lewandoski, Elizabeth Belloli, Alain Lescoat, Vivek Nagaraja, Wen Ye, Suiyuan Huang, Toby Maher, Dinesh Khanna
Summary: This study aimed to establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). The results showed that using the proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time.
Review
Biochemistry & Molecular Biology
Joe E. Mouawad, Carol Feghali-Bostwick
Summary: Systemic sclerosis, also known as scleroderma, is an autoimmune disorder that affects the connective tissues and has a high mortality rate. Fibrosis, particularly in the lungs, is a hallmark of the disease and is currently the leading cause of death. Understanding the molecular mechanisms involved in lung fibrosis is essential for developing potential therapies to improve patient outcomes and quality of life.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Tamas Nagy, Nora Melinda Toth, Erik Palmer, Lorinc Polivka, Balazs Csoma, Alexandra Nagy, Noemi Eszes, Krisztina Vincze, Eniko Barczi, Aniko Bohacs, Adam Domonkos Tarnoki, David Laszlo Tarnoki, Gyorgy Nagy, Emese Kiss, Pal Maurovich-Horvat, Veronika Muller
Summary: This study aimed to assess predictors of functional decline in SSc-ILD patients and compare disease behavior to IPF patients. The study found that cough and pulmonary hypertension are significant prognostic factors for functional progression in SSc-ILD patients.
Review
Rheumatology
David Roofeh, Alain Lescoat, Dinesh Khanna
Summary: The review provides an overview of current treatments for systemic sclerosis-interstitial lung disease (SSc-ILD) and proposes a conceptual framework for disease management. Treatment options include traditional cytotoxic therapies, biologic disease-modifying rheumatic drugs, antifibrotic agents, autologous hematopoietic stem cell transplant, and lung transplantation. The optimal use of each option varies depending on SSc-ILD severity, progression, and comorbidities.
CURRENT OPINION IN RHEUMATOLOGY
(2021)
Article
Medicine, General & Internal
Hye Jin Jang, Ala Woo, Song Yee Kim, Seung Hyun Yong, Youngmok Park, Kyungsoo Chung, Su Hwan Lee, Ah Young Leem, Sang Hoon Lee, Eun Young Kim, Ji Ye Jung, Young Ae Kang, Young Sam Kim, Moo Suk Park
Summary: This study aimed to identify risk factors for mortality and compare the clinical characteristics of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The study found that approximately 10% of SSc-ILD patients showed disease progression, and ILD was detected within less than five years from the first visit. Therefore, careful monitoring of symptoms and signs from an early stage and long-term surveillance are necessary.
ANNALS OF MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Veronica Pulito-Cueto, Sara Remuzgo-Martinez, Fernanda Genre, Belen Atienza-Mateo, Victor M. Mora-Cuesta, David Iturbe-Fernandez, Leticia Lera-Gomez, Raquel Perez-Fernandez, Diana Prieto-Pena, Virginia Portilla, Ricardo Blanco, Alfonso Corrales, Oreste Gualillo, Jose M. Cifrian, Raquel Lopez-Mejias, Miguel A. Gonzalez-Gay
Summary: Research has shown a significant increase in EPC frequency in SSc-ILD+ patients, which may be related to the pathogenic processes of vasculopathy and lung fibrosis. Additionally, EPC frequency was higher in male patients and negatively correlated with disease duration in SSc patients.
Article
Rheumatology
Marco Di Battista, Andrea Delle Sedie, Chiara Romei, Laura Tavanti, Mattia Da Rio, Riccardo Morganti, Alessandra Della Rossa, Marta Mosca
Summary: This study assessed the changes in lung ultrasound (LUS) and high-resolution CT (HRCT) in SSc-ILD patients treated with NIN, and found that NIN treatment significantly reduced the number of B-lines and pleural line irregularities. Male gender, smoking habit, and baseline forced vital capacity <70% predicted were associated with worse LUS outcomes. A greater reduction in imaging abnormalities was observed in those who improved in functional and quality-of-life outcomes.
Review
Medicine, General & Internal
Maria Martin-Lopez, Patricia E. Carreira
Summary: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis. Interstitial lung disease (ILD) is the leading cause of death in patients with SSc. The management of SSc-ILD has shifted to include targeted biologic and antifibrotic therapies.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Cell Biology
Qiuhong Wu, Yang Liu, Yan Xie, Shixiong Wei, Yi Liu
Summary: This study is the first to construct a potential ceRNA regulatory network and analyze the composition of infiltrating immune cells in lung tissues of SSc-ILD patients, which enhances our understanding of the pathogenesis of SSc-ILD.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Medicine, General & Internal
Wakar Garra, Yair Levy
Summary: This study aimed to estimate the prevalence of Anti-ARS antibodies among patients with systemic sclerosis and evaluate their clinical associations and risk factors for interstitial lung disease. The results showed a 6% prevalence of Anti-ARS antibodies, which were associated with the development of ILD.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2023)
Review
Medicine, General & Internal
Antoniya Kamenova, Argyris Tzouvelekis, George A. Margaritopoulos
Summary: Connective tissue diseases (CTDs) are a group of diverse systemic inflammatory disorders. Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a significant complication associated with high morbidity and mortality. This review explores the pathogenesis of CTD-ILD and summarizes recent clinical trial evidence for novel treatment options, including antifibrotics and immunomodulatory therapies, with a focus on systemic sclerosis-associated ILD. Ongoing clinical trials are investigating combination therapies and more targeted therapeutic approaches. Clinicians face the challenge of appropriately selecting patients for treatment and timing therapy initiation.
FRONTIERS IN MEDICINE
(2023)
Article
Rheumatology
Qiang Li, Laura Wallace, Padmaja Patnaik, Margarida Alves, Martina Gahlemann, Veronika Kohlbrenner, Christina Raabe, Jocelyn R. Wang, Elizabeth M. Garry
Summary: The study found low prevalence estimates and incidence rates for SSc and SSc-ILD in the US, with newly diagnosed SSc-ILD patients receiving more immunosuppressive therapy and having more comorbidities compared to newly diagnosed SSc patients.
Review
Biochemistry & Molecular Biology
Patricia Richter, Anca Cardoneanu, Nicoleta Dima, Ioana Bratoiu, Ciprian Rezus, Alexandra Maria Burlui, Damiana Costin, Luana Andreea Macovei, Elena Rezus
Summary: Interstitial lung disease (ILD) is a serious manifestation of connective tissue diseases (CTD) that requires thorough evaluation and treatment. The prevalence of ILD in systemic lupus erythematosus (SLE) is still debated, but it is crucial to exclude an overlap syndrome for an accurate diagnosis. Increasing the identification of SLE-associated ILD cases is important, and various treatment options are being proposed. Systemic sclerosis (SSc)-associated ILD is a leading cause of mortality and should be investigated in all SSc patients. Progress has been made in the treatment of ILD, with tyrosine kinases inhibitor Nintedanib showing promising results in reducing the progression rate compared to placebo.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Respiratory System
Marc Humbert, Vallerie McLaughlin, J. Simon R. Gibbs, Mardi Gomberg-Maitland, Marius M. Hoeper, Ioana R. Preston, Rogerio Souza, Aaron B. Waxman, Hossein-Ardeschir Ghofrani, Pilar Escribano Subias, Jeremy Feldman, Gisela Meyer, David Montani, Karen M. Olsson, Solaiappan Manimaran, Janethe de Oliveira Pena, David B. Badesch
Summary: In participants with pulmonary arterial hypertension, 24 weeks of sotatercept resulted in a significant reduction in pulmonary vascular resistance. This study reports the longer-term safety and efficacy of sotatercept in the PULSAR open-label extension, showing that sotatercept has good safety and efficacy in patients with pulmonary arterial hypertension.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Editorial Material
Respiratory System
Jason Weatherald, Marc Humbert
Article
Respiratory System
Hunter Gillies, Ralph Niven, Benjamin T. Dake, Murali M. Chakinala, Jeremy P. Feldman, Nicholas S. Hill, Marius M. Hoeper, Marc Humbert, Vallerie V. McLaughlin, Martin Kankam
Summary: AV-101, a dry powder inhaled formulation of imatinib, was well tolerated in healthy adults and significantly reduced the systemic exposure of imatinib compared with oral imatinib. An ongoing study will evaluate the safety, tolerability, and clinical benefit of AV-101 for pulmonary arterial hypertension.
Editorial Material
Critical Care Medicine
Jason Weatherald, Umberto Zanini, Marc Humbert
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Critical Care Medicine
Mithum Kularatne, Melanie Eyries, Laurent Savale, Marc Humbert, David Montani
Summary: Heritable pulmonary arterial hypertension (PAH) is often associated with pathogenic variants of BMPR2. This study describes two patients with pulmonary arteriovenous malformations (AVMs) who were found to carry variants of BMPR2 through gene sequencing. Through these cases and understanding of the BMP9 pathway, it is suggested that BMPR2 variants may increase the risk of pulmonary AVMs.
Article
Cardiac & Cardiovascular Systems
Jeremie Pichon, Anne Roche, Charles Fauvel, Athenais Boucly, Olaf Mercier, Nathan Ebstein, Antoine Beurnier, Jonathan Cortese, Mitja Jevnikar, Xavier Jais, Muriel Fartoukh, Elie Fadel, Olivier Sitbon, David Montani, Guillaume Voiriot, Marc Humbert, Laurent Savale
Summary: This study aimed to evaluate the clinical relevance and prognostic value of arterial and venous renal Doppler in acute decompensated precapillary pulmonary hypertension (PH). The results showed that the renal Doppler provides additional information to assess the severity and prognosis of patients with acute decompensated precapillary PH admitted to the intensive care unit.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2023)
Article
Cardiac & Cardiovascular Systems
Julien Grynblat, Sophie-Guiti Malekzadeh-Milani, Mathilde Meot, Frederic Perros, Isabelle Szezepanski, Stephane Morisset, Caroline Ovaert, Caroline Bonnet, Pascale Maragnes, Julien Ranchoup, Marc Humbert, I. David Montani, Marilyne Levy, Damien Bonnet
Summary: This study aims to define the goals of repeated right heart catheterization (RHC) and evaluate the correlation between noninvasive criteria and hemodynamic parameters. The results showed that pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure were associated with adverse outcomes in pediatric pulmonary arterial hypertension. Noninvasive criteria accurately predicted the evolution of hemodynamic parameters, but some patients still had at least one at-risk hemodynamic parameter at second RHC.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2023)
Editorial Material
Respiratory System
Vincent Cottin, Claudia Valenzuela, Marc Humbert
EUROPEAN RESPIRATORY JOURNAL
(2023)
Editorial Material
Medicine, General & Internal
Marc Humbert, Thomas Wagner
Review
Critical Care Medicine
Laurent Savale, Mithum Kularatne, Anne Roche, Jeremie Pichon, Audrey Baron, Athenais Boucly, Olivier Sitbon, Marc Humbert
Summary: Pulmonary arterial hypertension is a life-threatening condition characterized by increased pulmonary vascular resistance and right heart dysfunction. Admission to the intensive care unit with acute decompensated right heart failure is associated with a high risk of organ dysfunction and death. Various organ systems can be affected, and prompt identification and management of triggering events are crucial. Treatment goals include reversing ventriculo-arterial decoupling and reducing right ventricular afterload to prevent the progression of right heart failure. In cases of refractory dysfunction, lung transplantation and mechanical circulatory support may be considered.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Critical Care Medicine
Benoit Lechartier, Mithum Kularatne, Xavier Jais, Marc Humbert, David Montani
Summary: Pulmonary hypertension (PH) is a group of diseases characterized by abnormally elevated pulmonary arterial pressures diagnosed on right heart catheterization. The 2022 ESC/ERS Guidelines provide a new hemodynamic definition for PH, lowering the threshold of mean pulmonary artery pressure (mPAP) to 20 mm Hg. Precapillary PH is now defined as mPAP >20 mm Hg with normal pulmonary artery wedge pressure (<15 mm Hg) and increased pulmonary vascular resistance (>2 Wood Units). The guidelines also introduce a revised clinical classification of PH based on underlying pathophysiology.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Critical Care Medicine
Jason Weatherald, Rhea A. Varughese, Jonathan Liu, Marc Humbert
Summary: Pulmonary arterial hypertension (PAH) is a rare disease characterized by progressive pulmonary arterial remodeling, increased resistance, right ventricular dysfunction, and reduced survival. Current approved therapies for PAH target nitric oxide, endothelin-1, and prostacyclin pathways. Treatment includes oral medications for low- and intermediate-risk patients, with consideration of triple therapy for high-risk patients. Combination therapy may not be suitable for PAH patients with cardiopulmonary comorbidities. Supportive and adjunctive treatments are also important, and lung transplantation may be considered for patients who do not respond to medical care. Additional therapies are needed to improve outcomes in PAH.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Correction
Cardiac & Cardiovascular Systems
Hossein-Ardeschir Ghofrani, Ekkehard Grunig, Pavel Jansa, David Langleben, Stephan Rosenkranz, Ioana R. Preston, Franck Rahaghi, Namita Sood, Dennis Busse, Christian Meier, Marc Humbert
PULMONARY CIRCULATION
(2023)
Article
Respiratory System
Sharon Mumby, Frederic Perros, Julien Grynblat, Gregoire Manaud, Alberto Papi, Paolo Casolari, Gaetano Caramori, Marc Humbert, S. John Wort, Ian M. Adcock
Summary: In this study, elevated levels of BET proteins were found in pulmonary vascular cells of PAH patients, and JQ1+ was able to suppress TNF-α-induced inflammatory responses. These findings suggest that BET proteins could be a potential target for treating PAH.
RESPIRATORY RESEARCH
(2023)
Letter
Medicine, General & Internal
Jason Weatherald, Marc Humbert
