Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives

被引：906

作者：

Humbert, Marc ^{[1
,2
,3
,4
]}

Guignabert, Christophe ^{[1
,2
,3
]}

Bonnet, Sebastien ^{[5
,6
]}

Dorfmuller, Peter ^{[1
,2
,3
,7
]}

Klinger, James R. ^{[8
]}

Nicolls, Mark R. ^{[9
,10
,11
]}

Olschewski, Andrea J. ^{[12
,13
]}

Pullamsetti, Soni S. ^{[14
,15
]}

Schermuly, Ralph T. ^{[16
,17
,18
]}

Stenmark, Kurt R. ^{[19
]}

Rabinovitch, Marlene ^{[9
,10
,11
]}

机构：

[1] Univ Paris Sud, Fac Med, Le Kremlin Bicetre, France

[2] Univ Paris Saclay, Le Kremlin Bicetre, France

[3] INSERM, UMR S999, Le Plessis Robinson, France

[4] Hop Bicetre, AP HP, DHU,Ctr Reference Hypertens Pulm Severe, Thorax Innovat TORINO,Serv Pneumol, Le Kremlin Bicetre, France

[5] Ctr Rech Inst Cardiol & Pneumol Quebec, Pulm Hypertens Res Grp, Quebec City, PQ, Canada

[6] Univ Laval, Dept Med, Quebec City, PQ, Canada

[7] Hop Marie Lannelongue, Pathol Dept, Le Plessis Robinson, France

[8] Brown Univ, Rhode Isl Hosp, Warren Alpert Med Sch, Div Pulm Crit Care & Sleep Med,Dept Med, Providence, RI 02903 USA

[9] Stanford Univ, Sch Med, Dept Pediat, Cardiovasc Inst, 269 Campus Dr,CCSR Bldg,Room 1215A, Stanford, CA 94305 USA

[10] Stanford Univ, Sch Med, VA Palo Alto, Div Pulm & Crit Care Med,Dept Med, Palo Alto, CA 94304 USA

[11] Vera Moulton Wall Ctr Pulm Vasc Dis, Stanford, CA USA

[12] Ludwig Boltzmann Inst Lung Vasc Res, Graz, Austria

[13] Med Univ Graz, Inst Physiol, Graz, Austria

[14] Max Planck Inst Heart & Lung Res Bad Nauheim, Bad Nauheim, Germany

[15] Justus Liebig Univ Giessen, Excellence Cluster Cardio Pulm Inst CPI, Giessen, Germany

[16] Univ Giessen, Giessen, Germany

[17] Justus Liebig Univ Giessen, Marburg Lung Ctr UGMLC, Giessen, Germany

[18] German Ctr Lung Res DZL, Excellence Cluster Cardio Pulm Inst CPI, Giessen, Germany

[19] Univ Colorado, Dev Lung Biol & Cardiovasc Pulm Res Labs, Denver, CO 80202 USA

来源：

EUROPEAN RESPIRATORY JOURNAL | 2019年 / 53卷 / 01期

关键词：

TO-MESENCHYMAL TRANSITION; PROTEIN-RECEPTOR; 2; SMOOTH-MUSCLE-CELLS; GROWTH-FACTOR-BETA; ARTERIAL-HYPERTENSION; ENDOTHELIAL-CELLS; VASCULAR-LESIONS; ADVENTITIAL FIBROBLASTS; MITOCHONDRIAL-FUNCTION; MICROVASCULAR DISEASE;

D O I：

10.1183/13993003.01887-2018

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms of PH with severe vascular remodelling (e. g. chronic thromboembolic PH and pulmonary veno- occlusive disease). However, PH remains an incurable condition with a high mortality rate, underscoring the need for a better transfer of novel scientific knowledge into healthcare interventions. Herein, we review recent findings in pathology (with the questioning of the strict morphological categorisation of various forms of PH into pre- or post- capillary involvement of pulmonary vessels) and cellular mechanisms contributing to the onset and progression of pulmonary vascular remodelling associated with various forms of PH. We also discuss ways to improve management and to support and optimise drug development in this research field.

引用

页数：14

共 88 条

[61] Standards and Methodological Rigor in Pulmonary Arterial Hypertension Preclinical and Translational Research [J].

Provencher, Steeve ;

Archer, Stephen L. ;

Ramirez, F. Daniel ;

Hibbert, Benjamin ;

Paulin, Roxane ;

Boucherat, Olivier ;

Lacasse, Yves ;

Bonnet, Sebastien .

CIRCULATION RESEARCH, 2018, 122 (07) :1021-1032

[62] A Time- and Compartment-Specific Activation of Lung Macrophages in Hypoxic Pulmonary Hypertension [J].

Pugliese, Steven C. ;

Kumar, Sushil ;

Janssen, William J. ;

Graham, Brian B. ;

Frid, Maria G. ;

Riddle, Suzette R. ;

El Kasmi, Karim C. ;

Stenmark, Kurt R. .

JOURNAL OF IMMUNOLOGY, 2017, 198 (12) :4802-4812

[63] Lung cancer-associated pulmonary hypertension: Role of microenvironmental inflammation based on tumor cell-immune cell cross-talk [J].

Pullamsetti, Soni Savai ;

Kojonazarov, Baktybek ;

Storn, Samantha ;

Gall, Henning ;

Salazar, Ylia ;

Wolf, Janine ;

Weigert, Andreas ;

El-Nikhely, Nefertiti ;

Ghofrani, Hossein Ardeschir ;

Krombach, Gabriele A. ;

Fink, Ludger ;

Gattenlohner, Stefan ;

Rapp, Ulf R. ;

Schermuly, Ralph Theo ;

Grimminger, Friedrich ;

Seeger, Werner ;

Savai, Rajkumar .

SCIENCE TRANSLATIONAL MEDICINE, 2017, 9 (416)

[64] Leukotriene B4 Activates Pulmonary Artery Adventitial Fibroblasts in Pulmonary Hypertension [J].

Qian, Jin ;

Tian, Wen ;

Jiang, Xinguo ;

Tamosiuniene, Rasa ;

Sung, Yon K. ;

Shuffle, Eric M. ;

Tu, Allen B. ;

Valenzuela, Antonia ;

Jiang, Shirley ;

Zamanian, Roham T. ;

Fiorentino, David F. ;

Voelkel, Norbert F. ;

Peters-Golden, Marc ;

Stenmark, Kurt R. ;

Chung, Lorinda ;

Rabinovitch, Marlene ;

Nicolls, Mark R. .

HYPERTENSION, 2015, 66 (06) :1227-1239

[65] Endothelial-to-Mesenchymal Transition in Pulmonary Hypertension [J].

Ranchoux, Benoit ;

Antigny, Fabrice ;

Rucker-Martin, Catherine ;

Hautefort, Aurelie ;

Pechoux, Christine ;

Bogaard, Harm Jan ;

Dorfmueller, Peter ;

Remy, Severine ;

Lecerf, Florence ;

Plante, Sylvie ;

Chat, Sophie ;

Fadel, Elie ;

Houssaini, Amal ;

Anegon, Ignacio ;

Adnot, Serge ;

Simonneau, Gerald ;

Humbert, Marc ;

Cohen-Kaminsky, Sylvia ;

Perros, Frederic .

CIRCULATION, 2015, 131 (11) :1006-+

[66] Review DNA Damage and Pulmonary Hypertension [J].

Ranchoux, BenoOt ;

Meloche, Jolyane ;

Paulin, Roxane ;

Boucherat, Olivier ;

Provencher, Steeve ;

Bonnet, Sebastien .

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, 2016, 17 (06)

[67] RNA Sequencing Analysis Detection of a Novel Pathway of Endothelial Dysfunction in Pulmonary Arterial Hypertension [J].

Rhodes, Christopher J. ;

Im, Hogune ;

Cao, Aiqin ;

Hennigs, Jan K. ;

Wang, Lingli ;

Sa, Silin ;

Chen, Pin-I ;

Nickel, Nils P. ;

Miyagawa, Kazuya ;

Hopper, Rachel K. ;

Tojais, Nancy F. ;

Li, Caiyun G. ;

Gu, Mingxia ;

Spiekerkoetter, Edda ;

Xian, Zhaoying ;

Chen, Rui ;

Zhao, Mingming ;

Kaschwich, Mark ;

del Rosario, Patricia A. ;

Bernstein, Daniel ;

Zamanian, Roham T. ;

Wu, Joseph C. ;

Snyder, Michael P. ;

Rabinovitch, Marlene .

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2015, 192 (03) :356-366

[68] Increased Pericyte Coverage Mediated by-Endothelial-Derived Fibroblast Growth Factor-2 and Interleukin-6 Is a Source of Smooth Muscle-Like Cells in Pulmonary Hypertension [J].

Ricard, Nicolas ;

Tu, Ly ;

Le Hiress, Morane ;

Huertas, Alice ;

Phan, Carole ;

Thuillet, Raphael ;

Sattler, Caroline ;

Fadel, Elie ;

Seferian, Andrei ;

Montani, David ;

Dorfmueller, Peter ;

Humbert, Marc ;

Guignabert, Christophe .

CIRCULATION, 2014, 129 (15) :1586-1597

[69] Role for Runt-related Transcription Factor 2 in Proliferative and Calcified Vascular Lesions in Pulmonary Arterial Hypertension [J].

Ruffenach, Gregoire ;

Chabot, Sophie ;

Tanguay, Virginie F. ;

Courboulin, Audrey ;

Boucherat, Olivier ;

Potus, Francois ;

Meloche, Jolyane ;

Pflieger, Aude ;

Breuils-Bonnet, Sandra ;

Nadeau, Valerie ;

Paradis, Renee ;

Tremblay, Eve ;

Girerd, Barbara ;

Hautefort, Aurelie ;

Montani, David ;

Fadel, Elie ;

Dorfmuller, Peter ;

Humbert, Marc ;

Perros, Frederic ;

Paulin, Roxane ;

Provencher, Steeve ;

Bonnet, Sebastien .

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2016, 194 (10) :1273-1285

[70] Induced Pluripotent Stem Cell Model of Pulmonary Arterial Hypertension Reveals Novel Gene Expression and Patient Specificity [J].

Sa, Silin ;

Gu, Mingxia ;

Chappe, James ;

Shao, Ning-Yi ;

Ameen, Mohamed ;

Elliott, Kathryn A. T. ;

Li, Dan ;

Grubert, Fabian ;

Li, Caiyun G. ;

Taylor, Shalina ;

Cao, Aiqin ;

Ma, Yu ;

Fong, Ryan ;

Nguyen, Long ;

Wu, Joseph C. ;

Snyder, Michael P. ;

Rabinovitch, Marlene .

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2017, 195 (07) :930-941

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