Evidence map›Paper›PMID 39695595›Full record

ArticleRespiratory research2024

Diagnostic and prognostic implications of family history of fibrotic interstitial lung diseases.

D Duminy-Luppi, A Alcaide-Aldeano, L Planas-Cerezales, G Bermudo, V Vicens-Zygmunt, P Luburich, B Del Río-Carrero, R Llatjós, L Pijuan, I Escobar and 15 more

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In one paragraph

Article in Respiratory research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing 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

3 citing papers in PubMed.

  1. Review
  2. Review
  3. 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

25 authors.

D Duminy-LuppiUniversity of Barcelona, School of Medicine. Respiratory Department, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain. duminy.diego@gmail.com.
A Alcaide-AldeanoUniversity of Barcelona, School of Medicine. Respiratory Department, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain.
L Planas-CerezalesRespiratory Department, Hospital of Viladecans, IDIBELL, University of Barcelona, Viladecans, Spain.
G BermudoInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
V Vicens-ZygmuntInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
P LuburichRadiology Department, Bellvitge University Hospital, IDIBELL, University of Barcelona, L'Hospitalet de Llobregat, Spain.
B Del Río-CarreroRadiology Department, Bellvitge University Hospital, IDIBELL, University of Barcelona, L'Hospitalet de Llobregat, Spain.
R LlatjósPathology Department, Bellvitge University, L'Hospitalet de Llobregat, Spain.
L PijuanPathology Department, Bellvitge University, L'Hospitalet de Llobregat, Spain.
I EscobarDepartment of Thoracic Surgery, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain.
F RivasDepartment of Thoracic Surgery, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain.
A Montes-WorboysInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
Y Gutiérrez-RodríguezInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
D Rodríguez-PlazaInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
A Padró-MiquelDepartment of Clinical Genetics, Bellvitge University Hospital, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Spain.
A Esteve-GarciaDepartment of Clinical Genetics, Bellvitge University Hospital, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Spain.
B Fernández-VarasTelomeropathies Lab, CSIC-IIB Alberto Sols, Madrid, Spain.
C FloresCentro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain.
M FuentesInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain.
J DorcaUniversity of Barcelona, School of Medicine. Respiratory Department, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain.
S SantosUniversity of Barcelona, School of Medicine. Respiratory Department, Bellvitge University Hospital, L'Hospitalet de Llobregat, Spain.
R PeronaTelomeropathies Lab, CSIC-IIB Alberto Sols, Madrid, Spain.
A GüntherJustus Liebig University, Giessen, Germany.
J ShullBellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat, Spain.
M Molina-MolinaInterstitial Lung Disease Unit, Respiratory Department, Bellvitge University Hospital, University of Barcelona, L'Hospitalet de Llobregat, Spain. mariamolinamolina@hotmail.com.

Funding

Cabildo Insular de Tenerife CGIEU0000219140Instituto de Salud Carlos III and the European Joint Programme on Rare Diseases (EJP RD) AC19/00006Instituto Tecnológico y de Energías Renovables OA17/008 and OA23/043Ministerio de Ciencia e Innovación RTC-2017-6471-1; AEI/FEDER, UE
6 · The paper itself

Abstract

backgroundPatients with familial fibrotic interstitial lung disease (ILD) experience worse survival than patients with sporadic disease. Current guidelines do not consider family aggregation or genetic information in the diagnostic algorithm for idiopathic pulmonary fibrosis or other fibrotic ILDs. Better characterizing familial cases could help in diagnostic and treatment decision-making.

methodsThis retrospective cohort study included 222 patients with fibrotic ILD (104 familial and 118 sporadic) from Bellvitge University Hospital. Clinical, radiological, pulmonary functional tests (PFT), and histological evaluations were performed at diagnosis and follow-up. Telomere shortening and disease-associated variants (DAVs) in telomerase-related genes were analysed in familial patients and sporadic patients with telomeric clinical signs. Primary outcomes were the presence of a UIP histological pattern and disease progression.

resultsPatients with idiopathic pulmonary fibrosis (IPF) (52%), fibrotic hypersensitivity pneumonitis (23%), and other fibrotic ILDs (25%) were included. 42% of patients underwent lung biopsy. Patients with family aggregation were younger and less frequently associated comorbidities, male sex, and smoking history. However, usual interstitial pneumonia (UIP) was more frequent on pathology (p = 0.005; OR 3.37), especially in patients with indeterminate or non-UIP radiological patterns. Despite similar PFT results at diagnosis, familial patients were more likely to present with progressive disease (p = 0.001; OR 3.75). Carrying a DAV increased the risk of fibrotic progression in familial and sporadic patients (p = 0.029, OR 5.01). DISCUSSION: Familial patients diagnosed with different fibrotic ILDs were more likely to exhibit a histological UIP pattern and disease progression than sporadic patients, independent of radiological findings and pulmonary function at diagnosis.

conclusionConsidering the diagnostic likelihood of the histological UIP pattern and disease outcome, the presence of family aggregation would be useful in the decision making of multidisciplinary committees.

Indexed as

Lung Diseases, InterstitialAgedCohort StudiesDisease ProgressionFemaleFollow-Up StudiesHumansIdiopathic Pulmonary FibrosisMaleMiddle AgedPrognosisRetrospective StudiesBiopsyDisease ProgressionFamilyFibrosisIdiopathic pulmonary fibrosisLungLung diseases, interstitialPrognosisRetrospective studiesTelomere Shortening

Identifiers

PMID39695595
PMCPMC11656921

What Socratic holds

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LicenceCC BY-NC-ND
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Registered trials

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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 Socratic graph.