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

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

Publicated to:Frontiers of Medicine. 8 699644- - 2021-09-27 8(), DOI: 10.3389/fmed.2021.699644

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

Affiliations

Aarhus Univ Hosp, Dept Resp Dis & Allergy, Aarhus C, Denmark - Author

CHU Montreal, Dept Med, Montreal, PQ, Canada - Author

Ctr Invest Biomed Red Enfermedades Resp CIBERES, Madrid, Spain - Author

Erasmus Univ, Dept Resp Med, Ctr Interstitial Lung Dis & Sarcoidosis, Med Ctr, Rotterdam, Netherlands - Author

German Ctr Lung Res DZL, Marburg, Germany - Author

Heidelberg Univ, Ctr Interstitial & Rare Lung Dis, Pneumol, Thoraxklin, Heidelberg, Germany - Author

Heidelberg Univ, Inst Med Biometry & Informat, Heidelberg, Germany - Author

Hosp Britanico, Buenos Aires, DF, Argentina - Author

Imperial Coll London, Natl Heart & Lung Inst, Royal Brompton & Harefield NHS Fdn Trust, Interstitial Lung Dis Unit, London, England - Author

Imperial Coll, Natl Heart & Lung Inst, London, England - Author

Inst Nacl Enfermedades Resp Ismael Cosio Villegas, Mexico City, DF, Mexico - Author

Manchester Univ NHS Fdn Trust, North West Interstitial Lung Disease Unit, Manchester, Lancs, England - Author

McMaster Univ, Firestone Inst Resp Hlth, Res Inst St Josephs Healthcare, Dept Med, Hamilton, ON, Canada - Author

Ruhrlandklinik Univ Hosp Essen, Dept Pulm Med, Ctr Interstitial & Rare Lung Dis, Essen, Germany - Author

Sanatorio Guemes Hosp Privado, Buenos Aires, DF, Argentina - Author

Sechenov First Moscow State Med Univ, Moscow, Russia - Author

Tosei Gen Hosp, Dept Resp Med & Allergy, Seto, Japan - Author

Univ Athens, Sotiria Gen Hosp Thorac Dis, Dept Acad Resp 1, Athens, Greece - Author

Univ Autonoma Madrid, Hosp Univ La Princesa, Dept Pulmonol, ILD Unit, Madrid, Spain - Author

Univ Bern, Univ Hosp Bern, Dept Pulm Med, Inselspital, Bern, Switzerland - Author

Univ Calif San Francisco, Dept Med, San Francisco, CA 94143 USA - Author

Univ Cape Town, Div Pulmonol, Dept Med, Cape Town, South Africa - Author

Univ Catania, AOU Policlin Vittorio Emanuele, Reg Referral Ctr Rare Lung Dis, Catania, Italy - Author

Univ Claude Bernard Lyon 1, Louis Pradel Hosp, Hosp Civils Lyon, Natl Coordinating Reference Ctr Rare Pulm Dis, Lyon, France - Author

Univ Fed Sao Paulo, Paulista Sch Med, Lung Dis Dept, Sao Paulo, Brazil - Author

Univ Hosp Bellvitge, Inst Invest Biomed Bellvitge IDIBELL, Lhospitalet De Llobregat, Spain - Author

Univ Hosp Bellvitge, Pneumol Res Grp, Dept Pneumol, Unit Interstitial Lung Dis, Barcelona, Spain - Author

Univ Hosp Leuven, Dept Resp Dis, Unit Interstitial Lung Dis, Leuven, Belgium - Author

Univ Michigan, Dept Med, Ann Arbor, MI 48109 USA - Author

Univ Munich, Med Klin & Poliklin 5, LMU Klinikum, Munich, Germany - Author

Univ Porto, Ctr Hosp Sao Joao, Fac Med, Dept Pneumol, Porto, Portugal - Author

Univ Southern Calif, Hastings Ctr Pulm Res, Los Angeles, CA 90007 USA - Author

Univ Southern Calif, Keck Sch Med, Div Pulm Crit Care & Sleep Med, Los Angeles, CA 90007 USA - Author

Univ Sydney, Royal Prince Alfred Hosp, Sydney, NSW, Australia - Author

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 exacerbationidiopathic pulmonary fibrosisnon-specialised ild centrespulmonologistsquestionnaireAcute exacerbationControlled-trialCyclophosphamideDiagnosisHypertensionIdiopathic pulmonary fibrosisLung-diseaseNon-specialised ild centresOutcomesPirfenidonePulmonologistsQuestionnaireSpecialised ild centresSurvivalTherapy

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Bibliometric impact. Analysis of the contribution and dissemination channel

The work has been published in the journal Frontiers of Medicine due to its progression and the good impact it has achieved in recent years, according to the agency WoS (JCR), it has become a reference in its field. In the year of publication of the work, 2021, it was in position 32/245, thus managing to position itself as a Q1 (Primer Cuartil), in the category Oncology.

From a relative perspective, and based on the normalized impact indicator calculated from World Citations from Scopus Elsevier, it yields a value for the Field-Weighted Citation Impact from the Scopus agency: 1.12, which indicates that, compared to works in the same discipline and in the same year of publication, it ranks as a work cited above average. (source consulted: ESI Nov 14, 2024)

This information is reinforced by other indicators of the same type, which, although dynamic over time and dependent on the set of average global citations at the time of their calculation, consistently position the work at some point among the top 50% most cited in its field:

Field Citation Ratio (FCR) from Dimensions: 4.88 (source consulted: Dimensions Jun 2025)

Specifically, and according to different indexing agencies, this work has accumulated citations as of 2025-06-15, the following number of citations:

WoS: 6
Scopus: 10
OpenCitations: 9

Impact and social visibility

Leadership analysis of institutional authors

This work has been carried out with international collaboration, specifically with researchers from: Argentina; Australia; Belgium; Brazil; Canada; Denmark; France; Germany; Greece; Italy; Japan; Mexico; Netherlands; Portugal; Russia; South African Republic; Switzerland; United Kingdom; United States of America.

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