Evidence map›Paper›PMID 39901224›Full record

ArticleBMC pulmonary medicine2025

Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.

Emil Vilstrup Moen, Thomas Skovhus Prior, Michael Kreuter, Wim A Wuyts, Maria Molina-Molina, Marlies Wijsenbeek, Antonió Morais, Argyrios Tzouvelekis, Christopher J Ryerson, Fabian Caro and 14 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in BMC pulmonary medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

24 authors.

Emil Vilstrup MoenDepartment of Respiratory Diseases and Allergy, Centre for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark. emivil@rm.dk.
Thomas Skovhus PriorDepartment of Respiratory Diseases and Allergy, Centre for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark.
Michael KreuterLung Center Mainz, Department of Pneumology, Mainz University Medical Center and Department of Pulmonary, Critical Care & Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
Wim A WuytsUnit for Interstitial Lung Diseases, Dept Respiratory Medicine, University Hospitals Leuven, Leuven, Belgium.
Maria Molina-MolinaILD Unit, Respiratory Department, University Hospital of Bellvitge. IDIBELL. CIBERES, Barcelona, Spain.
Marlies WijsenbeekCentre for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus University Medical Centre, Rotterdam, The Netherlands.
Antonió MoraisPulmonology Department, Centro Hospitalar São João, Porto, Portugal Faculty of Medicine, University of Porto, Porto, Portugal i3S Instituto de Biologia Molecular E Celular/Instituto de Investigação E Inovação Em Saúde, University of Porto, Porto, Portugal.
Argyrios TzouvelekisDepartment of Respiratory Medicine, University of Patras, Patras, Greece.
Christopher J RyersonDepartment of Medicine and Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada.
Fabian CaroILD Unit, "Maria Ferrer" Hospital. Buenos Aires City, Buenos Aires, Argentina.
Ivette Buendia-RoldanLaboratory of Traslational Research in Aging and Fibrosis Lung Disease. Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico.
Jesper M MagnussonDepartement of Respiratory Medicine, Institute of Medicine, University of Gothenburg, Gothenburg, Sweden.
Joyce S LeeDepartment of Medicine, University of CO Denver - Anschutz Medical Campus, Aurora, CO, USA.
Julie MorisettDépartement de Médecine, Centre Hospitalier de L'Université de Montréal, Montréal, Québec, Canada.
Justin M OldhamDivision of Pulmonary and Critical Care Medicine, University of Michigan, Michigan, USA.
Lauren K TroyDepartment of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, Sydney, Australia.
Manuela Funke-ChambourDepartment for Pulmonary Medicine, Allergology and Clinical Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Maria Laura AlbertiILD Unit, "Maria Ferrer" Hospital. Buenos Aires, Buenos Aires, Argentina.
Raphael BorieService de Pneumologie A Hôpital Bichat, APHP, Paris, France, Université Paris Cité, Inserm, PHERE, Université Paris Cité, 75018, Paris, France.
Simon L F WalshNational Heart and Lung Institute Imperial College, London, England.
Sujeet RajanDepartment of Chest Medicine, Interstitial Lung Disease, Bombay Hospital Institute of Medical Sciences, and Bhatia Hospital, Mumbai, India.
Yasuhiro KondohDepartment of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
Yet H KhorRespiratory Research@Alfred, School of Translational Medicine, Monash University, Melbourne, VIC, Australia.
Elisabeth BendstrupDepartment of Respiratory Diseases and Allergy, Centre for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark.

Funding

Proteomic Profiling of Idiopathic Pulmonary Fibrosis Progression TrajectoryR01HL169166 · NHLBI · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Justin M Oldham · 2023 to 2026
$2.7M
Prognostic Biomarker Development in Progressive Fibrosing Interstitial Lung DiseaseR01HL166290 · NHLBI · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Justin M Oldham · 2024 to 2026
$2.2M
NHLBI NIH HHS R01 HL166290NHLBI NIH HHS R01 HL169166
6 · The paper itself

Abstract

backgroundAdvances in the field of genetics of interstitial lung diseases (ILDs) have led to the recent consensus statements made by expert groups. International standards for genetic testing in ILD have not yet been established. We aimed to examine current real-world strategies employed by pulmonologists working with familial ILD.

methodsA panel of pulmonologists with expertise in ILD developed an international survey aimed at clinicians working with ILD. The survey consisted of 74 questions divided into eight topics: characteristics of respondents, diagnosis, screening of first-degree relatives, screening tools, genetic testing methods, lung transplantation, ethical concerns, and future needs.

resultsOverall, 237 pulmonologists from 50 countries participated. A family history of ILD was asked for by 91% of respondents while fewer asked for symptoms related to telomere disorders. Respondents stated that 59% had access to genetic testing, and 30% to a genetic multidisciplinary team (MDT). Many respondents were unaware of specific genetic testing methods. Pathogenic genetic variants were seen as a potential contraindication for lung transplantation in 6-8% of respondents. Genetic screening of relatives was supported by 80% of respondents who indicated insufficient evidence and a lack of formal guidelines for genetics and ILD. Only 16% had a standardized program.

conclusionMost pulmonologists ask for a family history of ILD and recommend genetic testing for ILD and screening in relatives but have limited knowledge of specific tests and access to genetic MDT. Evidence-based guidelines to inform patients, relatives, and physicians are still warranted.

Indexed as

Genetic TestingLung Diseases, InterstitialPractice Patterns, Physicians'PulmonologistsFemaleHumansLung TransplantationMaleMiddle AgedSurveys and QuestionnairesFamilial ILDFamilial pulmonary fibrosisGenetic testingInternational surveyInterstitial lung diseaseMultidisciplinary team meetings

Identifiers

PMID39901224
PMCPMC11792556

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.