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The Mucin Gene MUC5B Is Required for Normal Lung Function
Maude A. Liegeois 1 and John V. Fahy 2,3
doi : 10.1164/rccm.202201-0064ED
Volume 205, Issue 7 | April 1 2022
Targeting Protease Activity to Interrupt Acute Respiratory Distress Syndrome Pathogenesis
Ciara M. Shaver
The P2X7 Receptor in Cystic Fibrosis Monocytes: Linking CFTR Deficiency to Inflammation
André M. Cantin 1,2
Fatum Inexorabile: Do Monocytes Predict the Fate of Interstitial Lung Abnormalities?
Michael Kreuter 1,2 and Toby M. Maher 3,4,5
More Evidence for Inflammatory Thrombosis in Chronic Thromboembolic Pulmonary Hypertension: Is the Embolic Hypothesis Losing Ground?
Smriti Sharma and Irene M. Lang
Is There a Role for Using Race-Specific Reference Equations? Yes and No
David A. Kaminsky
Are Bacille Calmette-Guérin Skin Reactions the Most Important Correlates of Bacille Calmette-Guérin’s Specific and Nonspecific Effects?
Frederik Schaltz-Buchholzer 1,2
Aggressive Afterload Lowering to Improve the Right Ventricle: A New Target for Medical Therapy in Pulmonary Arterial Hypertension?
Carmine Dario Vizza 1*, Irene M. Lang 2*, Roberto Badagliacca 1*, Raymond L. Benza 3*, Stephan Rosenkranz 4,5*, R. James White 6*, Yochai Adir 7,8, Arne K. Andreassen 9, Vijay Balasubramanian 10, Sonja Bartolome 11, Isabel Blanco 12,13, Robert C. Bourge 14, Jørn Carlsen 15,16, Rafael Enrique Conde Camacho 17, Michele D’Alto 18, Harrison W. Farber 19, Robert P. Frantz 20, H. James Ford 21, Stefano Ghio 22, Mardi Gomberg-Maitland 23, Marc Humbert 24,25,26, Robert Naeije 27, Stylianos E. Orfanos 28, Ronald J. Oudiz 29, Sergio V. Perrone 30, Oksana A. Shlobin 31, Marc A. Simon 32, Olivier Sitbon 24,25,26, Fernando Torres 11, Jean Luc Vachiery 33, Kuo-Yang Wang 34, Magdi H. Yacoub 35, Yan Liu 36, Gil Golden 36, and Hiromi Matsubara 37* ... Show less
doi : 10.1164/rccm.202109-2079PP
Despite numerous therapeutic advances in pulmonary arterial hypertension, patients continue to suffer high morbidity and mortality, particularly considering a median age of 50 years.
Hereditary Mucin Deficiency Caused by Biallelic Loss of Function of MUC5B
Gregory Costain 1,2,3*, Zhen Liu 3,4,5,6*, Vito Mennella 7, Giorgia Radicioni 8,9, Adrienn N. Goczi 4,5, Alexandra Albulescu 4,5, Susan Walker 3,10, Bo Ngan 11, David Manson 12,13, Reza Vali 12,13, Meraj Khan 14, Nades Palaniyar 14,15, David B. Hill 8,16, David A. Hall 17,18, Christian R. Marshall 10,15,19, Michael Knowles 8,20, Maimoona A. Zariwala 8,9, Mehmet Kesimer 8,9, and Sharon D. Dell 2,21,22,23 ... Show less
doi : 10.1164/rccm.202106-1456OC Â
Rationale: Mucin homeostasis is fundamental to airway health. Upregulation of airway mucus glycoprotein MUC5B is observed in diverse common lung diseases and represents a potential therapeutic target. In mice, Muc5b is required for mucociliary clearance and for controlling inflammation after microbial exposure. The consequences of its loss in humans are unclear.
Cathepsin S Contributes to Lung Inflammation in Acute Respiratory Distress Syndrome
Michael C. McKelvey 1, Anthony A. Abladey 1, Donna M. Small 1, Declan F. Doherty 1, Richard Williams 2, Aaron Scott 3, C. Arnold Spek 4, Keren S. Borensztajn 5†, Leslie Holsinger 6, Robert Booth 6, Cecilia M. O’Kane 7, Daniel F. McAuley 7, Clifford C. Taggart 1, and Sinéad Weldon 1
doi : 10.1164/rccm.202107-1631OC
Rationale: Although the cysteine protease cathepsin S has been implicated in the pathogenesis of several inflammatory lung diseases, its role has not been examined in the context of acute respiratory distress syndrome, a condition that still lacks specific and effective pharmacological treatments.
Trikafta Rescues CFTR and Lowers Monocyte P2X7R-induced Inflammasome Activation in Cystic Fibrosis
Claudie Gabillard-Lefort 1, Michelle Casey 1,2, Arlene M. A. Glasgow 3, Fiona Boland 4, Orla Kerr 2, Elaine Marron 2, Anne-Marie Lyons 2, Cedric Gunaratnam 1,2, Noel G. McElvaney 1,2, and Emer P. Reeves 1
doi : 10.1164/rccm.202106-1426OC
Rationale: Cystic fibrosis (CF) is caused by mutations in the CFTR (CF transmembrane conductance regulator) gene and is characterized by sustained inflammation. ATP triggers IL-1β secretion via P2X7R (P2X7 receptor) and activation of the NLRP3 (NOD-, LRR-, and pyrin domain–containing protein 3) inflammasome.
Associations of Monocyte Count and Other Immune Cell Types with Interstitial Lung Abnormalities
John S. Kim 1,2*, GÃÂsli Thor Axelsson 3,4*, Matthew Moll 5,6*, Michaela R. Anderson 2, Elana J. Bernstein 2, Rachel K. Putman 5, Tomoyuki Hida 7,8, Hiroto Hatabu 7, Eric A. Hoffman 9,10,11, Ganesh Raghu 12, Steven M. Kawut 13,14, Margaret F. Doyle 15, Russell Tracy 15, Lenore J. Launer 16, Ani Manichaikul 17, Stephen S. Rich 17, David J. Lederer 18, Vilmundur Gudnason 3,4, Brian D. Hobbs 5,6, Michael H. Cho 5,6, Gary M. Hunninghake 5, Christine Kim Garcia 2, Gunnar Gudmundsson 3,19, R. Graham Barr 2,20, and Anna J. Podolanczuk 2,21
doi : 10.1164/rccm.202108-1967OC
Rationale: Higher blood monocyte counts are associated with worse survival in adults with clinically diagnosed pulmonary fibrosis. Their association with the development and progression of interstitial lung abnormalities (ILA) in humans is unknown.
Epigenetic Modification of the von Willebrand Factor Promoter Drives Platelet Aggregation on the Pulmonary Endothelium in Chronic Thromboembolic Pulmonary Hypertension
Xue D. Manz 1, Robert Szulcek 1,2, Xiaoke Pan 1, Petr Symersky 3, Chris Dickhoff 3, Jisca Majolée 4, Veerle Kremer 4, Elisabetta Michielon 5, Ekaterina S. Jordanova 6, Teodora Radonic 7, Irene V. Bijnsdorp 8, Sander R. Piersma 8, Thang V. Pham 8, Connie R. Jimenez 8, Anton Vonk Noordegraaf 1, Frances S. de Man 1, Reinier A. Boon 4, Jan Voorberg 9, Peter L. Hordijk 4, Jurjan Aman 1*, and Harm Jan Bogaard 1*
doi : 10.1164/rccm.202109-2075OC
Rationale: von Willebrand factor (vWF) mediates platelet adhesion during thrombosis. While chronic thromboembolic pulmonary hypertension (CTEPH) is associated with increased plasma levels of vWF, the role of this protein in CTEPH has remained enigmatic.
Reconsidering the Utility of Race-Specific Lung Function Prediction Equations
Aaron D. Baugh 1, Stephen Shiboski 1, Nadia N. Hansel 2, Victor Ortega 3, Igor Barjaktarevic 4, R. Graham Barr 5, Russell Bowler 6, Alejandro P. Comellas 7, Christopher B. Cooper 2, David Couper 8, Gerard Criner 9, Jeffrey L. Curtis 10,11, Mark Dransfield 12, Chinedu Ejike 2, MeiLan K. Han 10, Eric Hoffman 7, Jamuna Krishnan 13, Jerry A. Krishnan 14, David Mannino 15, Robert Paine III16, Trisha Parekh 12, Stephen Peters 3, Nirupama Putcha 2, Stephen Rennard 17, Neeta Thakur 1*, and Prescott G. Woodruff 1*
doi : 10.1164/rccm.202105-1246OC
Rationale: African American individuals have worse outcomes in chronic obstructive pulmonary disease (COPD).
Bacillus Calmette-Guérin Skin Reaction Predicts Enhanced Mycobacteria-Specific T-Cell Responses in Infants: A Post Hoc Analysis of a Randomized Controlled Trial
Laure F. Pittet 1,2,3, Nora Fritschi 4,5, Marc Tebruegge 3,6,7, Binita Dutta 8, Susan Donath 3,9, Nicole L. Messina 2,3, Dan Casalaz 10, Willem A. Hanekom 11, Warwick J. Britton 12, Roy Robins-Browne 2,13, Nigel Curtis 1,2,3*, Nicole Ritz 3,4,5*; and the BCG Immune Response Study (BIRS) Group
doi : 10.1164/rccm.202108-1892OC
Rationale: Scar formation following bacillus Calmette-Guérin (BCG) vaccination has been associated with lower all-cause mortality; the relation between scar and mycobacteria-specific protection against tuberculosis is debated.
Bronchotracheal Compression Caused by Esophageal Impaction after Bilateral Lung Transplantation
Aleksandra C. Zoethout 1, Michiel E. Erasmus 2, Erik Verschuuren 3, and Janesh Pillay 1,4
From the Cava to the Chest: Partial Anomalous Pulmonary Venous Connection
Hillary R. Johnson 1, Allison B. Couillard 2, Rebecca A. Busch 3, and Micah T. Long 4
A Randomized Clinical Trial Using CoronaVac or BNT162b2 Vaccine as a Third Dose in Adults Vaccinated with Two Doses of CoronaVac
Chris Ka Pun Mok 1, 2*, Chunke Chen 1, 2, Karen Yiu 2, Tat-On Chan 2, Kiu Cheung Lai 2, Kwun Cheung Ling 2, Yuanxin Sun 1, 2, David S. Hui 2‡, Samuel M. S. Cheng 3*, and Malik Peiris 3‡§
Awake Extracorporeal Membrane Oxygenation for COVID-19–induced Acute Respiratory Distress Syndrome
Sebastian Mang 1*, Christian Reyher 2*, Haitham Mutlak 3, Ruslan Natanov 4, Christopher Lotz 5, Daniel Gill-Schuster 3, Robert Bals 1, Guy Danziger 1, Patrick Meybohm 5, Alain Combes 6, Christian Kühn 4, Philipp M. Lepper 1*‡, and Ralf M. Muellenbach 2*; the AWECO-Study Group
Remote 6-Minute-Walk Testing in Patients with Pulmonary Hypertension: A Pilot Study
Tess LaPatra 1, Grayson L. Baird 2,3, Randi Goodman 1, Diane Pinder 1, Maeve Gaffney 3, James R. Klinger 3, Harold I. Palevsky 1, Jason Fritz 1, Christopher J. Mullin 3, Jeremy A. Mazurek 1, Steven M. Kawut 1*, and Corey E. Ventetuolo 3*‡
A Physiological Hypothesis to Support the Use of Continuous Positive Airway Pressure at Extubation among Patients with Obesity
Gaetano Florio 1,2, David A. Imber 1,2, and Lorenzo Berra 1,2*
Reply to Florio et al.: A Physiological Hypothesis to Support the Use of Continuous Positive Airway Pressure at Extubation among Patients with Obesity
Arnaud W. Thille 1,2*, Rémi Coudroy 1,2, Jean-Pierre Frat 1,2, and Stéphanie Ragot 2
MZ Alpha-1 Antitrypsin Deficiency
Oliver J McElvaney , Gerald Finnerty , Tomás P Carroll , Mark L Brantly , and Noel G McElvaney
doi : 10.1164/rccm.2057P13 Â
