A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey

Michael Kreuter; Markus Polke; Simon Walsh; Harold R Collard; Nazia Chaudhuri; Sergey Avdeev; Juergen Behr; Greg Calligero; Tamera Corte; Kevin Flaherty; Manuela Funke; Martin Kolb; Yasuhiro Kondoh; Toby M. Maher; Maria Molina Molina; Antonio Morais; Karen Moor; Julie Morisset; Carlos Pereira; Silvia Quadrelli; Moises Selman; Argyrios Tzouvelekis; Carlo Vancheri; Vanesa Vicens-Zygmunt; Julia Waelscher; Wim Wuyts; Marlies Wijsenbeek; Vincent Cottin; Elisabeth Bendstrup

doi:10.1183/13993003.congress-2018.OA542

A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey

Michael Kreuter
, Markus Polke
, Simon Walsh
, Harold R Collard
, Nazia Chaudhuri
, Sergey Avdeev
, Juergen Behr
, Greg Calligero
, Tamera Corte
, Kevin Flaherty
, Manuela Funke
, Martin Kolb
, Yasuhiro Kondoh
, Toby M. Maher
, Maria Molina Molina
, Antonio Morais
, Karen Moor
, Julie Morisset
, Carlos Pereira
, Silvia Quadrelli

Moises Selman, Argyrios Tzouvelekis, Carlo Vancheri, Vanesa Vicens-Zygmunt, Julia Waelscher, Wim Wuyts, Marlies Wijsenbeek, Vincent Cottin, Elisabeth Bendstrup

Research output: Contribution to journal › Conference article › peer-review

Abstract

Background: AE-IPF is a deadly complication of IPF, for which no international guidelines exist, resulting in global variability in prevention, diagnosis and treatment strategies.

Methods: Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.

Results: 469 pulmonologists responded (66 countries, 64% experts). Significant geographical variability in approach to managing AE-IPF was found (figure). Common preventive measures include antifibrotics and antacids. Diagnostic differences are most pronounced regarding use of KL-6 and viral testing, while HRCT, BNP and d-dimer are broadly used. High dose steroids are widely administered (92%), but use of immunosuppressant and other strategies is highly variable (fig). Very few (4%) responders never use immunosuppression. Antifibrotics are initiated during AE-IPF by 66%. Invasive ventilation or ECMO are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (63%).

Conclusion: International approaches to the prevention, diagnosis and treatment of AE-IPF are diverse. Global international guidelines and trials to evaluate these approaches are needed.

Original language	English
Article number	OA542
Journal	European Respiratory Journal
Volume	52
Issue number	Suppl 62
DOIs	https://doi.org/10.1183/13993003.congress-2018.OA542
Publication status	Published (in print/issue) - 15 Sept 2018

Bibliographical note

This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).

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10.1183/13993003.congress-2018.OA542

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Kreuter, M., Polke, M., Walsh, S., Collard, H. R., Chaudhuri, N., Avdeev, S., Behr, J., Calligero, G., Corte, T., Flaherty, K., Funke, M., Kolb, M., Kondoh, Y., Maher, T. M., Molina, M. M., Morais, A., Moor, K., Morisset, J., Pereira, C., ... Bendstrup, E. (2018). A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey. European Respiratory Journal, 52(Suppl 62), Article OA542. https://doi.org/10.1183/13993003.congress-2018.OA542

@article{47646165546a461f9ccfcc11686c010c,

title = "A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey",

abstract = "Background: AE-IPF is a deadly complication of IPF, for which no international guidelines exist, resulting in global variability in prevention, diagnosis and treatment strategies. Methods: Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. Results: 469 pulmonologists responded (66 countries, 64\% experts). Significant geographical variability in approach to managing AE-IPF was found (figure). Common preventive measures include antifibrotics and antacids. Diagnostic differences are most pronounced regarding use of KL-6 and viral testing, while HRCT, BNP and d-dimer are broadly used. High dose steroids are widely administered (92\%), but use of immunosuppressant and other strategies is highly variable (fig). Very few (4\%) responders never use immunosuppression. Antifibrotics are initiated during AE-IPF by 66\%. Invasive ventilation or ECMO are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82\%) and consider palliative care (63\%). Conclusion: International approaches to the prevention, diagnosis and treatment of AE-IPF are diverse. Global international guidelines and trials to evaluate these approaches are needed.",

author = "Michael Kreuter and Markus Polke and Simon Walsh and Collard, \{Harold R\} and Nazia Chaudhuri and Sergey Avdeev and Juergen Behr and Greg Calligero and Tamera Corte and Kevin Flaherty and Manuela Funke and Martin Kolb and Yasuhiro Kondoh and Maher, \{Toby M.\} and Molina, \{Maria Molina\} and Antonio Morais and Karen Moor and Julie Morisset and Carlos Pereira and Silvia Quadrelli and Moises Selman and Argyrios Tzouvelekis and Carlo Vancheri and Vanesa Vicens-Zygmunt and Julia Waelscher and Wim Wuyts and Marlies Wijsenbeek and Vincent Cottin and Elisabeth Bendstrup",

note = "This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).",

year = "2018",

month = sep,

day = "15",

doi = "10.1183/13993003.congress-2018.OA542",

language = "English",

volume = "52",

journal = "European Respiratory Journal",

issn = "0903-1936",

publisher = "European Respiratory Society",

number = "Suppl 62",

}

Kreuter, M, Polke, M, Walsh, S, Collard, HR, Chaudhuri, N, Avdeev, S, Behr, J, Calligero, G, Corte, T, Flaherty, K, Funke, M, Kolb, M, Kondoh, Y, Maher, TM, Molina, MM, Morais, A, Moor, K, Morisset, J, Pereira, C, Quadrelli, S, Selman, M, Tzouvelekis, A, Vancheri, C, Vicens-Zygmunt, V, Waelscher, J, Wuyts, W, Wijsenbeek, M, Cottin, V & Bendstrup, E 2018, 'A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey', European Respiratory Journal, vol. 52, no. Suppl 62, OA542. https://doi.org/10.1183/13993003.congress-2018.OA542

TY - JOUR

T1 - A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey

AU - Kreuter, Michael

AU - Polke, Markus

AU - Walsh, Simon

AU - Collard, Harold R

AU - Chaudhuri, Nazia

AU - Avdeev, Sergey

AU - Behr, Juergen

AU - Calligero, Greg

AU - Corte, Tamera

AU - Flaherty, Kevin

AU - Funke, Manuela

AU - Kolb, Martin

AU - Kondoh, Yasuhiro

AU - Maher, Toby M.

AU - Molina, Maria Molina

AU - Morais, Antonio

AU - Moor, Karen

AU - Morisset, Julie

AU - Pereira, Carlos

AU - Quadrelli, Silvia

AU - Selman, Moises

AU - Tzouvelekis, Argyrios

AU - Vancheri, Carlo

AU - Vicens-Zygmunt, Vanesa

AU - Waelscher, Julia

AU - Wuyts, Wim

AU - Wijsenbeek, Marlies

AU - Cottin, Vincent

AU - Bendstrup, Elisabeth

N1 - This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).

PY - 2018/9/15

Y1 - 2018/9/15

N2 - Background: AE-IPF is a deadly complication of IPF, for which no international guidelines exist, resulting in global variability in prevention, diagnosis and treatment strategies. Methods: Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. Results: 469 pulmonologists responded (66 countries, 64% experts). Significant geographical variability in approach to managing AE-IPF was found (figure). Common preventive measures include antifibrotics and antacids. Diagnostic differences are most pronounced regarding use of KL-6 and viral testing, while HRCT, BNP and d-dimer are broadly used. High dose steroids are widely administered (92%), but use of immunosuppressant and other strategies is highly variable (fig). Very few (4%) responders never use immunosuppression. Antifibrotics are initiated during AE-IPF by 66%. Invasive ventilation or ECMO are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (63%). Conclusion: International approaches to the prevention, diagnosis and treatment of AE-IPF are diverse. Global international guidelines and trials to evaluate these approaches are needed.

AB - Background: AE-IPF is a deadly complication of IPF, for which no international guidelines exist, resulting in global variability in prevention, diagnosis and treatment strategies. Methods: Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. Results: 469 pulmonologists responded (66 countries, 64% experts). Significant geographical variability in approach to managing AE-IPF was found (figure). Common preventive measures include antifibrotics and antacids. Diagnostic differences are most pronounced regarding use of KL-6 and viral testing, while HRCT, BNP and d-dimer are broadly used. High dose steroids are widely administered (92%), but use of immunosuppressant and other strategies is highly variable (fig). Very few (4%) responders never use immunosuppression. Antifibrotics are initiated during AE-IPF by 66%. Invasive ventilation or ECMO are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (63%). Conclusion: International approaches to the prevention, diagnosis and treatment of AE-IPF are diverse. Global international guidelines and trials to evaluate these approaches are needed.

U2 - 10.1183/13993003.congress-2018.OA542

DO - 10.1183/13993003.congress-2018.OA542

M3 - Conference article

SN - 0903-1936

VL - 52

JO - European Respiratory Journal

JF - European Respiratory Journal

IS - Suppl 62

M1 - OA542

ER -

A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey

Abstract

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