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Published in: Annals of Intensive Care 1/2020

01-12-2020 | Idiopathic Pulmonary Fibrosis | Review

Ventilatory support and mechanical properties of the fibrotic lung acting as a “squishy ball”

Authors: Alessandro Marchioni, Roberto Tonelli, Giulio Rossi, Paolo Spagnolo, Fabrizio Luppi, Stefania Cerri, Elisabetta Cocconcelli, Maria Rosaria Pellegrino, Riccardo Fantini, Luca Tabbì, Ivana Castaniere, Lorenzo Ball, Manu L. N. G. Malbrain, Paolo Pelosi, Enrico Clini

Published in: Annals of Intensive Care | Issue 1/2020

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Abstract

Protective ventilation is the cornerstone of treatment of patients with the acute respiratory distress syndrome (ARDS); however, no studies have yet established the best ventilatory strategy to adopt when patients with acute exacerbation of interstitial lung disease (AE-ILD) are admitted to the intensive care unit. Due to the severe impairment of the respiratory mechanics, the fibrotic lung is at high risk of developing ventilator-induced lung injury, regardless of the lung fibrosis etiology. The purpose of this review is to analyze the effects of mechanical ventilation in AE-ILD and to increase the knowledge on the characteristics of fibrotic lung during artificial ventilation, introducing the concept of “squishy ball lung”. The role of positive end-expiratory pressure is discussed, proposing a “lung resting strategy” as opposed to the “open lung approach”. The review also discusses the practical management of AE-ILD patients discussing illustrative clinical cases.
Literature
1.
go back to reference Travis WD, Costabel U, Hansell DM, King TE, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–48.PubMedPubMedCentralCrossRef Travis WD, Costabel U, Hansell DM, King TE, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–48.PubMedPubMedCentralCrossRef
2.
go back to reference Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet. 2012;380:689–98.PubMedCrossRef Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet. 2012;380:689–98.PubMedCrossRef
3.
go back to reference Marchioni A, Tonelli R, Ball L, Fantini R, Castaniere I, Cerri S, et al. Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome? Crit Care. 2018;22:80.PubMedPubMedCentralCrossRef Marchioni A, Tonelli R, Ball L, Fantini R, Castaniere I, Cerri S, et al. Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome? Crit Care. 2018;22:80.PubMedPubMedCentralCrossRef
4.
go back to reference Tachikawa R, Tomii K, Ueda H, Nagata K, Nanjo S, Sakurai A, et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration. 2012;83:20–7.PubMedCrossRef Tachikawa R, Tomii K, Ueda H, Nagata K, Nanjo S, Sakurai A, et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration. 2012;83:20–7.PubMedCrossRef
5.
go back to reference Upagupta C, Shimbori C, Alsilmi R, Kolb M. Matrix abnormalities in pulmonary fibrosis. Eur Respir Rev. 2018;27:180033.PubMedCrossRef Upagupta C, Shimbori C, Alsilmi R, Kolb M. Matrix abnormalities in pulmonary fibrosis. Eur Respir Rev. 2018;27:180033.PubMedCrossRef
7.
8.
go back to reference Gattinoni L, Marini JJ, Pesenti A, Quintel M, Mancebo J, Brochard L. The, “baby lung” became an adult. Intensive Care Med. 2016;42:663–73.PubMedCrossRef Gattinoni L, Marini JJ, Pesenti A, Quintel M, Mancebo J, Brochard L. The, “baby lung” became an adult. Intensive Care Med. 2016;42:663–73.PubMedCrossRef
9.
go back to reference Kuhn C, Boldt J, King TE, Crouch E, Vartio T, McDonald JA. An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis. Am Rev Respir Dis. 1989;140:1693–703.PubMedCrossRef Kuhn C, Boldt J, King TE, Crouch E, Vartio T, McDonald JA. An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis. Am Rev Respir Dis. 1989;140:1693–703.PubMedCrossRef
10.
go back to reference Plantier L, Cazes A, Dinh-Xuan A-T, Bancal C, Marchand-Adam S, Crestani B. Physiology of the lung in idiopathic pulmonary fibrosis. Eur Respir Rev. 2018;27:170062.PubMedCrossRef Plantier L, Cazes A, Dinh-Xuan A-T, Bancal C, Marchand-Adam S, Crestani B. Physiology of the lung in idiopathic pulmonary fibrosis. Eur Respir Rev. 2018;27:170062.PubMedCrossRef
11.
go back to reference Lambert RK, Paré PD. Lung parenchymal shear modulus, airway wall remodeling, and bronchial hyperresponsiveness. J Appl Physiol. 1997;83:140–7.PubMedCrossRef Lambert RK, Paré PD. Lung parenchymal shear modulus, airway wall remodeling, and bronchial hyperresponsiveness. J Appl Physiol. 1997;83:140–7.PubMedCrossRef
12.
go back to reference Chiumello D, Carlesso E, Cadringher P, Caironi P, Valenza F, Polli F, et al. Lung stress and strain during mechanical ventilation for acute respiratory distress syndrome. Am J Respir Crit Care Med. 2008;178:346–55.PubMedCrossRef Chiumello D, Carlesso E, Cadringher P, Caironi P, Valenza F, Polli F, et al. Lung stress and strain during mechanical ventilation for acute respiratory distress syndrome. Am J Respir Crit Care Med. 2008;178:346–55.PubMedCrossRef
13.
go back to reference Cressoni M, Cadringher P, Chiurazzi C, Amini M, Gallazzi E, Marino A, et al. Lung inhomogeneity in patients with acute respiratory distress syndrome. Am J Respir Crit Care Med. 2014;189:149–58.PubMed Cressoni M, Cadringher P, Chiurazzi C, Amini M, Gallazzi E, Marino A, et al. Lung inhomogeneity in patients with acute respiratory distress syndrome. Am J Respir Crit Care Med. 2014;189:149–58.PubMed
14.
15.
go back to reference Güngör G, Tatar D, Saltürk C, Çimen P, Karakurt Z, Kirakli C, et al. Why do patients with interstitial lung diseases fail in the ICU? A 2-center cohort study. Respir Care. 2013;58:525–31.PubMedCrossRef Güngör G, Tatar D, Saltürk C, Çimen P, Karakurt Z, Kirakli C, et al. Why do patients with interstitial lung diseases fail in the ICU? A 2-center cohort study. Respir Care. 2013;58:525–31.PubMedCrossRef
16.
go back to reference Fernández-Pérez ER, Yilmaz M, Jenad H, Daniels CE, Ryu JH, Hubmayr RD, et al. Ventilator settings and outcome of respiratory failure in chronic interstitial lung disease. Chest. 2008;133:1113–9.PubMedCrossRef Fernández-Pérez ER, Yilmaz M, Jenad H, Daniels CE, Ryu JH, Hubmayr RD, et al. Ventilator settings and outcome of respiratory failure in chronic interstitial lung disease. Chest. 2008;133:1113–9.PubMedCrossRef
17.
go back to reference Moua T, Westerly BD, Dulohery MM, Daniels CE, Ryu JH, Lim KG. Patients with fibrotic interstitial lung disease hospitalized for acute respiratory worsening: a large cohort analysis. Chest. 2016;149:1205–14.PubMedCrossRef Moua T, Westerly BD, Dulohery MM, Daniels CE, Ryu JH, Lim KG. Patients with fibrotic interstitial lung disease hospitalized for acute respiratory worsening: a large cohort analysis. Chest. 2016;149:1205–14.PubMedCrossRef
18.
go back to reference Zafrani L, Lemiale V, Lapidus N, Lorillon G, Schlemmer B, Azoulay E. Acute respiratory failure in critically ill patients with interstitial lung disease. PLoS ONE. 2014;9:e104897.PubMedPubMedCentralCrossRef Zafrani L, Lemiale V, Lapidus N, Lorillon G, Schlemmer B, Azoulay E. Acute respiratory failure in critically ill patients with interstitial lung disease. PLoS ONE. 2014;9:e104897.PubMedPubMedCentralCrossRef
19.
go back to reference Grasselli G, Vergnano B, Pozzi MR, Sala V, D’Andrea G, Scaravilli V, et al. Interstitial pneumonia with autoimmune features: an additional risk factor for ARDS? Ann Intensive Care. 2017;7:98.PubMedPubMedCentralCrossRef Grasselli G, Vergnano B, Pozzi MR, Sala V, D’Andrea G, Scaravilli V, et al. Interstitial pneumonia with autoimmune features: an additional risk factor for ARDS? Ann Intensive Care. 2017;7:98.PubMedPubMedCentralCrossRef
20.
go back to reference Sahetya SK, Goligher EC, Brower RG. Setting positive end-expiratory pressure in acute respiratory distress syndrome. Care Med: Am J Respir Crit; 2017. Sahetya SK, Goligher EC, Brower RG. Setting positive end-expiratory pressure in acute respiratory distress syndrome. Care Med: Am J Respir Crit; 2017.
21.
go back to reference Kacmarek RM, Villar J, Sulemanji D, Montiel R, Ferrando C, Blanco J, et al. Open lung approach for the acute respiratory distress syndrome: a pilot, randomized controlled trial. Crit Care Med. 2016;44:32–42.PubMedCrossRef Kacmarek RM, Villar J, Sulemanji D, Montiel R, Ferrando C, Blanco J, et al. Open lung approach for the acute respiratory distress syndrome: a pilot, randomized controlled trial. Crit Care Med. 2016;44:32–42.PubMedCrossRef
22.
go back to reference Fujino Y, Goddon S, Dolhnikoff M, Hess D, Amato MBP, Kacmarek RM. Repetitive high-pressure recruitment maneuvers required to maximally recruit lung in a sheep model of acute respiratory distress syndrome. Crit Care Med. 2001;29:1579–86.PubMedCrossRef Fujino Y, Goddon S, Dolhnikoff M, Hess D, Amato MBP, Kacmarek RM. Repetitive high-pressure recruitment maneuvers required to maximally recruit lung in a sheep model of acute respiratory distress syndrome. Crit Care Med. 2001;29:1579–86.PubMedCrossRef
23.
go back to reference Cavalcanti AB, Suzumura ÉA, Laranjeira LN, De Moraes Paisani D, Damiani LP, Guimarães HP, et al. Effect of lung recruitment and titrated Positive End-Expiratory Pressure (PEEP) vs low PEEP on mortality in patients with acute respiratory distress syndrome—a randomized clinical trial. JAMA. 2017;318:1335–45.PubMedPubMedCentralCrossRef Cavalcanti AB, Suzumura ÉA, Laranjeira LN, De Moraes Paisani D, Damiani LP, Guimarães HP, et al. Effect of lung recruitment and titrated Positive End-Expiratory Pressure (PEEP) vs low PEEP on mortality in patients with acute respiratory distress syndrome—a randomized clinical trial. JAMA. 2017;318:1335–45.PubMedPubMedCentralCrossRef
24.
go back to reference Pelosi P, Rocco PR, de Abreu MG. Close down the lungs and keep them resting to minimize ventilator-induced lung injury. Critical Care. 2018;2(1):72.CrossRef Pelosi P, Rocco PR, de Abreu MG. Close down the lungs and keep them resting to minimize ventilator-induced lung injury. Critical Care. 2018;2(1):72.CrossRef
25.
go back to reference Cruz FF, Ball L, Rocco PRM, Pelosi P. Ventilator-induced lung injury during controlled ventilation in patients with acute respiratory distress syndrome: less is probably better. Expert Rev Respir Med. 2018;12:403–14.PubMedCrossRef Cruz FF, Ball L, Rocco PRM, Pelosi P. Ventilator-induced lung injury during controlled ventilation in patients with acute respiratory distress syndrome: less is probably better. Expert Rev Respir Med. 2018;12:403–14.PubMedCrossRef
26.
27.
go back to reference Akoumianaki E, Maggiore SM, Valenza F, Bellani G, Jubran A, Loring SH, et al. The application of esophageal pressure measurement in patients with respiratory failure. Am J Respir Crit Care Med. 2014;189:520–31.PubMedCrossRef Akoumianaki E, Maggiore SM, Valenza F, Bellani G, Jubran A, Loring SH, et al. The application of esophageal pressure measurement in patients with respiratory failure. Am J Respir Crit Care Med. 2014;189:520–31.PubMedCrossRef
28.
go back to reference Yoshida T, Amato MBP, Grieco DL, Chen L, Lima CAS, Roldan R, et al. Esophageal manometry and regional transpulmonary pressure in lung injury. Am J Respir Crit Care Med. 2018;197:1018–26.PubMedCrossRef Yoshida T, Amato MBP, Grieco DL, Chen L, Lima CAS, Roldan R, et al. Esophageal manometry and regional transpulmonary pressure in lung injury. Am J Respir Crit Care Med. 2018;197:1018–26.PubMedCrossRef
29.
go back to reference Mietto C, Malbrain MLNG, Chiumello D. Transpulmonary pressure monitoring during mechanical ventilation: a bench-to-bedside review. Anestezjol Intens Ter. 2015;47:27–37. Mietto C, Malbrain MLNG, Chiumello D. Transpulmonary pressure monitoring during mechanical ventilation: a bench-to-bedside review. Anestezjol Intens Ter. 2015;47:27–37.
30.
go back to reference Talmor D, Sarge T, Malhotra A, O’Donnell CR, Ritz R, Lisbon A, et al. Mechanical ventilation guided by esophageal pressure in acute lung injury. N Engl J Med. 2008;359:2095–104.PubMedPubMedCentralCrossRef Talmor D, Sarge T, Malhotra A, O’Donnell CR, Ritz R, Lisbon A, et al. Mechanical ventilation guided by esophageal pressure in acute lung injury. N Engl J Med. 2008;359:2095–104.PubMedPubMedCentralCrossRef
31.
go back to reference Beitler JR, Sarge T, Banner-Goodspeed VM, Gong MN, Cook D, Novack V, et al. Effect of titrating positive end-expiratory pressure (PEEP) with an esophageal pressure-guided strategy vs an empirical high PEEP-Fio 2 strategy on death and days free from mechanical ventilation among patients with acute respiratory distress syndrome: a randomized clinical trial. JAMA. 2019;321:846–57.PubMedPubMedCentralCrossRef Beitler JR, Sarge T, Banner-Goodspeed VM, Gong MN, Cook D, Novack V, et al. Effect of titrating positive end-expiratory pressure (PEEP) with an esophageal pressure-guided strategy vs an empirical high PEEP-Fio 2 strategy on death and days free from mechanical ventilation among patients with acute respiratory distress syndrome:  a randomized clinical trial. JAMA. 2019;321:846–57.PubMedPubMedCentralCrossRef
32.
go back to reference Walkey AJ, Del Sorbo L, Hodgson CL, Adhikari NKJ, Wunsch H, Meade MO, et al. Higher PEEP versus lower PEEP strategies for patients with acute respiratory distress syndrome: a systematic review and meta-analysis. Ann Am Thorac Soc. 2017;14:S297–303.PubMedCrossRef Walkey AJ, Del Sorbo L, Hodgson CL, Adhikari NKJ, Wunsch H, Meade MO, et al. Higher PEEP versus lower PEEP strategies for patients with acute respiratory distress syndrome: a systematic review and meta-analysis. Ann Am Thorac Soc. 2017;14:S297–303.PubMedCrossRef
33.
go back to reference Park IN, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132(1):214–20.PubMedCrossRef Park IN, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132(1):214–20.PubMedCrossRef
34.
go back to reference Miyazaki Y, Tateishi T, Akashi T, Ohtani Y, Inase N, Yoshizawa Y. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest. 2008;134:1265–70.PubMedCrossRef Miyazaki Y, Tateishi T, Akashi T, Ohtani Y, Inase N, Yoshizawa Y. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest. 2008;134:1265–70.PubMedCrossRef
35.
go back to reference Rice AJ, Wells AU, Bouros D, du Bois RM, Hansell DM, Polychronopoulos V, et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study. Am J Clin Pathol. 2003;119:709–14.PubMedCrossRef Rice AJ, Wells AU, Bouros D, du Bois RM, Hansell DM, Polychronopoulos V, et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study. Am J Clin Pathol. 2003;119:709–14.PubMedCrossRef
36.
go back to reference Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–68.PubMedCrossRef Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–68.PubMedCrossRef
37.
go back to reference Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27:143–50.PubMedCrossRef Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27:143–50.PubMedCrossRef
38.
go back to reference Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner society: glossary of terms for thoracic imaging. Radiology. 2008;246:697–722.CrossRefPubMed Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner society: glossary of terms for thoracic imaging. Radiology. 2008;246:697–722.CrossRefPubMed
39.
go back to reference Ryerson CJ, Cottin V, Brown KK, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015;46:512–20.PubMedCrossRef Ryerson CJ, Cottin V, Brown KK, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015;46:512–20.PubMedCrossRef
40.
go back to reference Ball L, Sutherasan Y, Pelosi P. Monitoring respiration: what the clinician needs to know. Best Pract Res Clin Anaesthesiol. 2013;27:209–23.PubMedCrossRef Ball L, Sutherasan Y, Pelosi P. Monitoring respiration: what the clinician needs to know. Best Pract Res Clin Anaesthesiol. 2013;27:209–23.PubMedCrossRef
41.
go back to reference Brower RG, Matthay MA, Morris A, Schoenfeld D, Thompson BT, et al. Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome network. N Engl J Med. 2000;342:1301–8.PubMedCrossRef Brower RG, Matthay MA, Morris A, Schoenfeld D, Thompson BT, et al. Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome network. N Engl J Med. 2000;342:1301–8.PubMedCrossRef
42.
Metadata
Title
Ventilatory support and mechanical properties of the fibrotic lung acting as a “squishy ball”
Authors
Alessandro Marchioni
Roberto Tonelli
Giulio Rossi
Paolo Spagnolo
Fabrizio Luppi
Stefania Cerri
Elisabetta Cocconcelli
Maria Rosaria Pellegrino
Riccardo Fantini
Luca Tabbì
Ivana Castaniere
Lorenzo Ball
Manu L. N. G. Malbrain
Paolo Pelosi
Enrico Clini
Publication date
01-12-2020
Publisher
Springer International Publishing
Published in
Annals of Intensive Care / Issue 1/2020
Electronic ISSN: 2110-5820
DOI
https://doi.org/10.1186/s13613-020-0632-6

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