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

Frontiers in Medicine

Maria Molina Molina¹  Michael Kreuter²  Jürgen Behr²  Vanesa Vicens-Zygmunt³  Antonio Morais⁴  Julia Wälscher⁵  Gregory Calligaro⁶  Julie Morisset⁷  Tamera J. Corte⁸  Wim Wuyts⁹  Kevin Flaherty^1,10  Elisabeth Bendstrup^1,11  Carlos Pereira^1,12  Manuela Funke-Chambour^1,13  Martin Kolb^1,15  Silvia Quadrelli^1,16  Johannes Krisam^1,17  Moises Selman^1,18  Argyrios Tzouvelekis^1,19  Claudia Valenzuela^2,21  Toby M. Maher^2,22  Carlo Vancheri^2,24  Markus Polke^2,25  Harold R. Collard^2,26  Yasuhiro Kondoh^2,27  Marlies Wijsenbeek^2,28  Catharina C. Moor^2,28  Simon L. F. Walsh^2,29  Vincent Cottin^3,31  Nazia Chaudhuri^3,32  Sergey Avdeev^3,33 

[1] 0Centro de Investigación Biomédica en Red Enfermedades Respiratorias (CIBERES), Madrid, Spain;0German Center for Lung Research (DZL), Marburg, Germany;0Unit of Interstitial Lung Diseases, Department of Pneumology, Pneumology Research Group, IDIBELL, L'Hospitalet de Llobregat, University Hospital of Bellvitge, Barcelona, Spain;1Department of Pneumology, Faculdade de Medicina, Centro Hospitalar São João, Universidade do Porto, Porto, Portugal;1Department of Pulmonary Medicine, Centre for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital Essen, Essen, Germany;1Division of Pulmonology, Department of Medicine, University of Cape Town, Cape Town, South Africa;2Département de Médecine, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada;2Royal Prince Alfred Hospital, University of Sydney, Sydney, NSW, Australia;2Unit for Interstitial Lung Diseases, Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium;3Department of Medicine, University of Michigan, Ann Arbor, MI, United States;3Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus C, Denmark;3Lung Disease Department, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil;4Department of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;4Hospital Británico, Buenos Aires, Argentina;5Department of Medicine, Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, McMaster University, Hamilton, ON, Canada;5Sanatorio Güemes, Buenos Aires, Argentina;6Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany;6Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico;7Department of First Academic Respiratory, Sotiria General Hospital for Thoracic Diseases, University of Athens, Athens, Greece;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;8ILD Unit, Pulmonology Department Hospital Universitario de La Princesa, Universidad Autonoma de Madrid, Madrid, Spain;8Interstitial Lung Disease Unit, Imperial College London, National Heart and Lung Institute, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom;9Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), University Hospital of Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain;9Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy;Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany;Department of Medicine, University of California, San Francisco, San Francisco, CA, United States;Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan;Department of Respiratory Medicine, Centre for Interstitial Lung Diseases and Sarcoidosis, Erasmus University Medical Centre, Rotterdam, Netherlands;Imperial College, National Heart and Lung Institute, London, United Kingdom;Medizinische Klinik und Poliklinik V, LMU Klinikum, University of Munich, Munich, Germany;National Coordinating Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon 1, Lyon, France;North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe, Manchester, United Kingdom;Sechenov First Moscow State Medical University, Moscow, Russia;

关键词: idiopathic pulmonary fibrosis;    acute exacerbation;    questionnaire;    pulmonologists;    specialised ILD centres;    non-specialised ILD centres;   

DOI  :  10.3389/fmed.2021.699644

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown