Observational studyBMC pulmonary medicine2019
Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
Observational study in BMC pulmonary medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02951416 (The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank), which is not on this map. Cited by 31 papers, 1 of them a synthesis that pooled it.
What it found
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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.
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.
The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank)
Who cites it
31 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.American journal of respiratory and critical care medicine · 2022Guideline
- Investigation of telomere related gene mutations in idiopathic pulmonary fibrosis.Molecular biology reports · 2020Trial
- Geographical, institutional and workplace variability in genetic evaluation, screening practices, multidisciplinary care, and lung transplantation management for familial interstitial lung disease.BMC pulmonary medicine · 2026Article
- The Genetic Landscape of Fibrotic Interstitial Lung Diseases: Clinical Implications and Diagnostic Challenges in Familial Pulmonary Fibrosis.Journal of clinical medicine · 2026Review
- Clinical and genetic features of idiopathic pulmonary fibrosis in a Korean cohort.Respiratory research · 2026Article
- Bronchoalveolar lavage cell differential count is associated with survival and radiological UIP pattern in familial pulmonary fibrosis.BMC pulmonary medicine · 2026Article
- Concomitant Idiopathic Pulmonary Fibrosis and Lung Cancer: An Updated Narrative Review.Advances in respiratory medicine · 2025Review
- [Target organ the lungs: diversity of fibrotic pulmonary diseases].Innere Medizin (Heidelberg, Germany) · 2025Review
- Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.BMC pulmonary medicine · 2025Article
- Acute Exacerbation of Idiopathic Pulmonary Fibrosis in the Setting of Acute Eosinophilic Pneumonia.HCA healthcare journal of medicine · 2025Review
- Protocol and research program of the European registry and biobank for interstitial lung diseases (eurILDreg).BMC pulmonary medicine · 2024Article
- Review
- Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis.Respiratory research · 2024Article
- Article
- Emerging delivery approaches for targeted pulmonary fibrosis treatment.Advanced drug delivery reviews · 2024Review
- Genetic Underpinnings of Pulmonary Fibrosis: An Overview.Cardiovascular & hematological agents in medicinal chemistry · 2024Review
- Use of Next-Generation Sequencing to Support the Diagnosis of Familial Interstitial Pneumonia.Genes · 2023Article
- Optimizing Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS): A Prospective Cohort Study Design.Journal of clinical medicine · 2023Article
- Genomic Fingerprint Associated with Familial Idiopathic Pulmonary Fibrosis: A Review.International journal of medical sciences · 2023Review
- Genetic and environmental factors in interstitial lung diseases: current and future perspectives on early diagnosis of high-risk cohorts.Frontiers in medicine · 2023Article
Corrections and comments
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Authors and funding
11 authors.
Funding
Abstract
backgroundThe aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).
methodsFamilial IPF index patients and their family members were recruited into the European IPF registry/biobank (eurIPFreg) at the Universities of Giessen and Marburg (UGMLC). Initially, we employed wide range criteria of f-IPF (e.g. relatives who presumably died of some kind of parenchymal lung disease). After narrowing down the search to occurrence of idiopathic interstitial pneumonia (IIP) in at least one first grade relative, 28 index patients were finally identified, prospectively interviewed and examined. Their family members were phenotyped with establishment of pedigree charts.
resultsWithin the 28 IPF families, overall 79 patients with f-IPF were identified. In the same observation period, 286 f-IIP and s-IIP patients were recruited into the eurIPFreg at our UGMLC sites, corresponding to a familial versus s-IPF of 9.8%. The both groups showed no difference in demographics (61 vs. 79% males), smoking history, and exposure to any environmental triggers known to cause lung fibrosis. The f-IPF group differed by an earlier age at the onset of the disease (55.4 vs. 63.2 years; p < 0.001). On average, the f-IPF patients presented a significantly milder extent of functional impairment at the time point of inclusion vs. the s-IPF group (FVC 75% pred. vs. FVC 62% pred., p = 0.011). In contrast, the decline in FVC was found to be faster in the f-IPF vs. the s-IPF group (4.94% decline in 6 months in f-IPF vs. 2.48% in s-IPF, p = 0.12). The average age of death in f-IPF group was 67 years vs. 71.8 years in s-IPF group (p = 0.059). The f-IIP group displayed diverse inheritance patterns, mostly autosomal-dominant with variable penetrance. In the f-IPF, the younger generations showed a tendency for earlier manifestation of IPF vs. the older generation (58 vs. 66 years, p = 0.013).
conclusionsThe 28 f-IPF index patients presented an earlier onset and more aggressive natural course of the disease. The disease seems to affect consecutive generations at a younger age.
trial registrationNr. NCT02951416 http://www.www.clinicaltrials.gov.
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