ReviewChest2021
Familial Pulmonary Fibrosis: Genetic Features and Clinical Implications.
Review in Chest, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 27 papers.
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.
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.
Precision Diagnosis and Care for Families With Pulmonary Fibrosis in Ireland [PRECISE-PF]
An Observational, Prospective, Multicenter Study on Hereditary Influences on Pulmonary Fibrosis Trajectories
Who cites it
27 citing papers in PubMed.
- Telomere dysfunction and mucociliary impairment drive idiopathic pulmonary fibrosis susceptibility: insights from a Sardinian whole-exome study.BMJ open respiratory research · 2026Article
- Allicin Attenuates Silica-Induced Pulmonary Fibrosis by Targeting the Serpinb2/NF-κB Pathway.Journal of biochemical and molecular toxicology · 2026Article
- Murine models of pulmonary fibrosis: mechanisms, limitations and translational insights.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Advances in our understanding of distal progenitors in idiopathic pulmonary fibrosis: implications for novel therapeutics.The European respiratory journal · 2026Review
- Article
- Idiopathic Pulmonary Fibrosis: Analysis of Predisposing Variants in Patients with Familial Forms.Biomedicines · 2026Article
- Familial interstitial lung disease: emerging insights into screening and genetic risk.Frontiers in medicine · 2026Review
- Genetic Determinants of Progressive Pulmonary Fibrosis: A Comprehensive Review.International journal of molecular sciences · 2025Review
- DNA damage response and cell fate decisions across the lifespan: from fetal development to age-related respiratory diseases.Cell & bioscience · 2025Review
- Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis.Respirology (Carlton, Vic.) · 2025Article
- Updates in Diagnostic Tools for ILD.Journal of clinical medicine · 2025Review
- Endothelin receptor antagonists target EDNRB and modulate the progression of idiopathic pulmonary fibrosis via anoikis-related genes.Frontiers in medicine · 2025Article
- Diagnostic and prognostic implications of family history of fibrotic interstitial lung diseases.Respiratory research · 2024Article
- Article
- The management of Familial Pulmonary Fibrosis in different medical settings: Where does that leave us? An Italian nationwide survey.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis.Respiratory research · 2024Article
- Article
- Using Families to Study Pulmonary Fibrosis Genetics.American journal of respiratory and critical care medicine · 2023Article
- Defining the Genetic Landscape of Idiopathic Pulmonary Fibrosis: Role of Common and Rare Variants.American journal of respiratory and critical care medicine · 2023Article
- Analysis of influencing factors and a predictive model of small airway dysfunction in adults.BMC pulmonary medicine · 2023Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors.
Funding
Abstract
Pulmonary fibrosis comprises a wide range of fibrotic lung diseases with unknown pathogenesis and poor prognosis. Familial pulmonary fibrosis (FPF) represents a unique subgroup of patients in which at least one other relative is also affected. Patients with FPF exhibit a wide range of pulmonary fibrosis phenotypes, although idiopathic pulmonary fibrosis is the most common subtype. Despite variable disease manifestations, patients with FPF experience worse survival compared with their counterparts with the sporadic disease form. Therefore, ascertaining a positive family history not only provides prognostic value but should also raise suspicion for the inheritance of an underlying causative genetic variant within kindreds. By focusing on FPF kindreds, rare variants within surfactant metabolism and telomere maintenance genes have been discovered. However, such genetic variation is not solely restricted to FPF, as similar rare variants are found in patients with seemingly sporadic pulmonary fibrosis, further supporting the idea of genetic susceptibility underlying pulmonary fibrosis as a whole. Researchers are beginning to show how the presence of rare variants may inform clinical management, such as informing predisposition risk for yet unaffected relatives as well as informing prognosis and therapeutic strategy for those already affected. Despite these advances, rare variants in surfactant and telomere-related genes only explain the genetic basis in about one-quarter of FPF kindreds. Therefore, research is needed to identify the missing genetic contributors of pulmonary fibrosis, which would not only improve our understanding of disease pathobiology but may offer additional opportunities to improve the health of patients.
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What OpenQuestion holds
Registered trials
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.