Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World

Markus Polke; Yasuhiro Kondoh; Marlies Wijsenbeek; Vincent Cottin; Simon L F Walsh; Harold R Collard; Nazia Chaudhuri; Sergey Avdeev; Jürgen Behr; Gregory Calligaro; Tamera J Corte; Kevin Flaherty; Manuela Funke-Chambour; Martin Kolb; Johannes Krisam; Toby M Maher; Maria Molina Molina; Antonio Morais; Catharina C Moor; Julie Morisset; Carlos Pereira; Silvia Quadrelli; Moises Selman; Argyrios Tzouvelekis; Claudia Valenzuela; Carlo Vancheri; Vanesa Vicens-Zygmunt; Julia Wälscher; Wim Wuyts; Elisabeth Bendstrup; Michael Kreuter

doi:10.3389/fmed.2021.699644

Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World

Front Med (Lausanne). 2021 Sep 27:8:699644. doi: 10.3389/fmed.2021.699644. eCollection 2021.

Authors

Markus Polke¹, Yasuhiro Kondoh², Marlies Wijsenbeek³, Vincent Cottin⁴, Simon L F Walsh⁵, Harold R Collard⁶, Nazia Chaudhuri⁷, Sergey Avdeev⁸, Jürgen Behr^{9

10}, Gregory Calligaro¹¹, Tamera J Corte¹², Kevin Flaherty¹³, Manuela Funke-Chambour¹⁴, Martin Kolb¹⁵, Johannes Krisam¹⁶, Toby M Maher^{17

18}, Maria Molina Molina^{19

20}, Antonio Morais²¹, Catharina C Moor³, Julie Morisset²², Carlos Pereira²³, Silvia Quadrelli^{24

25}, Moises Selman²⁶, Argyrios Tzouvelekis²⁷, Claudia Valenzuela²⁸, Carlo Vancheri²⁹, Vanesa Vicens-Zygmunt³⁰, Julia Wälscher^{1

31}, Wim Wuyts³², Elisabeth Bendstrup³³, Michael Kreuter^{1

10}

Affiliations

¹ Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
² Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
³ Department of Respiratory Medicine, Centre for Interstitial Lung Diseases and Sarcoidosis, Erasmus University Medical Centre, Rotterdam, Netherlands.
⁴ National Coordinating Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon 1, Lyon, France.
⁵ Imperial College, National Heart and Lung Institute, London, United Kingdom.
⁶ Department of Medicine, University of California, San Francisco, San Francisco, CA, United States.
⁷ North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe, Manchester, United Kingdom.
⁸ Sechenov First Moscow State Medical University, Moscow, Russia.
⁹ Medizinische Klinik und Poliklinik V, LMU Klinikum, University of Munich, Munich, Germany.
¹⁰ German Center for Lung Research (DZL), Marburg, Germany.
¹¹ Division of Pulmonology, Department of Medicine, University of Cape Town, Cape Town, South Africa.
¹² Royal Prince Alfred Hospital, University of Sydney, Sydney, NSW, Australia.
¹³ Department of Medicine, University of Michigan, Ann Arbor, MI, United States.
¹⁴ Department of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
¹⁵ Department of Medicine, Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, McMaster University, Hamilton, ON, Canada.
¹⁶ Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany.
¹⁷ Hastings Centre for Pulmonary Research and Division of Pulmonary, Critical Care, and Sleep Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States.
¹⁸ Interstitial Lung Disease Unit, Imperial College London, National Heart and Lung Institute, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.
¹⁹ Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), University Hospital of Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.
²⁰ Centro de Investigación Biomédica en Red Enfermedades Respiratorias (CIBERES), Madrid, Spain.
²¹ Department of Pneumology, Faculdade de Medicina, Centro Hospitalar São João, Universidade do Porto, Porto, Portugal.
²² Département de Médecine, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada.
²³ Lung Disease Department, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil.
²⁴ Hospital Británico, Buenos Aires, Argentina.
²⁵ Sanatorio Güemes, Buenos Aires, Argentina.
²⁶ Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico.
²⁷ Department of First Academic Respiratory, Sotiria General Hospital for Thoracic Diseases, University of Athens, Athens, Greece.
²⁸ ILD Unit, Pulmonology Department Hospital Universitario de La Princesa, Universidad Autonoma de Madrid, Madrid, Spain.
²⁹ Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy.
³⁰ Unit of Interstitial Lung Diseases, Department of Pneumology, Pneumology Research Group, IDIBELL, L'Hospitalet de Llobregat, University Hospital of Bellvitge, Barcelona, Spain.
³¹ Department of Pulmonary Medicine, Centre for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital Essen, Essen, Germany.
³² Unit for Interstitial Lung Diseases, Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.
³³ Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus C, Denmark.

Abstract

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide. Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians' institutions. Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods. Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.

Keywords: acute exacerbation; idiopathic pulmonary fibrosis; non-specialised ILD centres; pulmonologists; questionnaire; specialised ILD centres.

Copyright © 2021 Polke, Kondoh, Wijsenbeek, Cottin, Walsh, Collard, Chaudhuri, Avdeev, Behr, Calligaro, Corte, Flaherty, Funke-Chambour, Kolb, Krisam, Maher, Molina Molina, Morais, Moor, Morisset, Pereira, Quadrelli, Selman, Tzouvelekis, Valenzuela, Vancheri, Vicens-Zygmunt, Wälscher, Wuyts, Bendstrup and Kreuter.