Frontiers in Medicine (Sep 2021)
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,
- Jürgen Behr,
- Gregory Calligaro,
- Tamera J. Corte,
- Kevin Flaherty,
- Manuela Funke-Chambour,
- Martin Kolb,
- Johannes Krisam,
- Toby M. Maher,
- Toby M. Maher,
- Maria Molina Molina,
- Maria Molina Molina,
- Antonio Morais,
- Catharina C. Moor,
- Julie Morisset,
- Carlos Pereira,
- Silvia Quadrelli,
- Silvia Quadrelli,
- Moises Selman,
- Argyrios Tzouvelekis,
- Claudia Valenzuela,
- Carlo Vancheri,
- Vanesa Vicens-Zygmunt,
- Julia Wälscher,
- Julia Wälscher,
- Wim Wuyts,
- Elisabeth Bendstrup,
- Michael Kreuter,
- Michael Kreuter
Affiliations
- Markus Polke
- Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
- Yasuhiro Kondoh
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan
- Marlies Wijsenbeek
- Department of Respiratory Medicine, Centre for Interstitial Lung Diseases and Sarcoidosis, Erasmus University Medical Centre, Rotterdam, Netherlands
- Vincent Cottin
- National Coordinating Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon 1, Lyon, France
- Simon L. F. Walsh
- Imperial College, National Heart and Lung Institute, London, United Kingdom
- Harold R. Collard
- Department of Medicine, University of California, San Francisco, San Francisco, CA, United States
- Nazia Chaudhuri
- North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe, Manchester, United Kingdom
- Sergey Avdeev
- Sechenov First Moscow State Medical University, Moscow, Russia
- Jürgen Behr
- Medizinische Klinik und Poliklinik V, LMU Klinikum, University of Munich, Munich, Germany
- Jürgen Behr
- 0German Center for Lung Research (DZL), Marburg, Germany
- Gregory Calligaro
- 1Division of Pulmonology, Department of Medicine, University of Cape Town, Cape Town, South Africa
- Tamera J. Corte
- 2Royal Prince Alfred Hospital, University of Sydney, Sydney, NSW, Australia
- Kevin Flaherty
- 3Department of Medicine, University of Michigan, Ann Arbor, MI, United States
- Manuela Funke-Chambour
- 4Department of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
- Martin Kolb
- 5Department of Medicine, Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, McMaster University, Hamilton, ON, Canada
- Johannes Krisam
- 6Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany
- Toby M. Maher
- 7Hastings 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
- Toby M. Maher
- 8Interstitial Lung Disease Unit, Imperial College London, National Heart and Lung Institute, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
- Maria Molina Molina
- 9Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), University Hospital of Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain
- Maria Molina Molina
- 0Centro de Investigación Biomédica en Red Enfermedades Respiratorias (CIBERES), Madrid, Spain
- Antonio Morais
- 1Department of Pneumology, Faculdade de Medicina, Centro Hospitalar São João, Universidade do Porto, Porto, Portugal
- Catharina C. Moor
- Department of Respiratory Medicine, Centre for Interstitial Lung Diseases and Sarcoidosis, Erasmus University Medical Centre, Rotterdam, Netherlands
- Julie Morisset
- 2Département de Médecine, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada
- Carlos Pereira
- 3Lung Disease Department, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil
- Silvia Quadrelli
- 4Hospital Británico, Buenos Aires, Argentina
- Silvia Quadrelli
- 5Sanatorio Güemes, Buenos Aires, Argentina
- Moises Selman
- 6Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico
- Argyrios Tzouvelekis
- 7Department of First Academic Respiratory, Sotiria General Hospital for Thoracic Diseases, University of Athens, Athens, Greece
- Claudia Valenzuela
- 8ILD Unit, Pulmonology Department Hospital Universitario de La Princesa, Universidad Autonoma de Madrid, Madrid, Spain
- Carlo Vancheri
- 9Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy
- Vanesa Vicens-Zygmunt
- 0Unit of Interstitial Lung Diseases, Department of Pneumology, Pneumology Research Group, IDIBELL, L'Hospitalet de Llobregat, University Hospital of Bellvitge, Barcelona, Spain
- Julia Wälscher
- Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
- Julia Wälscher
- 1Department of Pulmonary Medicine, Centre for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital Essen, Essen, Germany
- Wim Wuyts
- 2Unit for Interstitial Lung Diseases, Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium
- Elisabeth Bendstrup
- 3Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus C, Denmark
- Michael Kreuter
- Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
- Michael Kreuter
- 0German Center for Lung Research (DZL), Marburg, Germany
- DOI
- https://doi.org/10.3389/fmed.2021.699644
- Journal volume & issue
-
Vol. 8
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
- idiopathic pulmonary fibrosis
- acute exacerbation
- questionnaire
- pulmonologists
- specialised ILD centres
- non-specialised ILD centres
