ArticleSarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG2024
The management of Familial Pulmonary Fibrosis in different medical settings: Where does that leave us? An Italian nationwide survey.
Article in Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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13 citing papers in PubMed.
- The Genetic Landscape of Fibrotic Interstitial Lung Diseases: Clinical Implications and Diagnostic Challenges in Familial Pulmonary Fibrosis.Journal of clinical medicine · 2026Review
- Anti-cyclic citrullinated peptide antibody in bronchoalveolar lavage fluid of patients with idiopathic pulmonary fibrosis.BMC pulmonary medicine · 2026Article
- Rubiadin-1-methyl ether alleviates bleomycin induced pulmonary fibrosis.Scientific reports · 2026Article
- Clinical profiles and risk predictors of drug-induced liver injury from pirfenidone and nintedanib used to treat idiopathic pulmonary fibrosis: a real-world analysis of a tertiary Chinese hospital cohort combined with FAERS data mining.Translational gastroenterology and hepatology · 2026Article
- Sepsis related Brugada syndrome in community acquired pneumonia due to Legionella pneumophila: a case report.BMC pulmonary medicine · 2025Article
- Survival impact and safety comparison of pirfenidone and nintedanib for idiopathic pulmonary fibrosis: A meta-analysis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2025Article
- Retrospective Observational Study of Nintedanib in Managing Idiopathic and Progressive Pulmonary Fibrosis in Routine Practice.Journal of clinical medicine · 2025Article
- Clinical Characteristics and Survival of Patients with Idiopathic Pulmonary Fibrosis: Analysis of the Serbian Cohort from the EMPIRE Registry.Diagnostics (Basel, Switzerland) · 2025Article
- The Relationship Between Chest Wall Muscle Thickness, Pulmonary Function, and Prognostic Markers in Idiopathic Pulmonary Fibrosis.Medicina (Kaunas, Lithuania) · 2025Article
- Usual Interstitial Pneumonia Pattern and Mycobacteria Lung Diseases: A Case Series.Infectious disease reports · 2025Article
- Article
- Observational
- Exploring the Causal Relationship Between Saliva Microbiota Abundance and Chronic Obstructive Pulmonary Disease/Idiopathic Pulmonary Fibrosis: A Two-Sample Mendelian Randomization Study.International journal of chronic obstructive pulmonary disease · 2025Article
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12 authors.
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Abstract
BACKGROUND AND
aimFamilial Pulmonary Fibrosis (FPF) is an emerging group of interstitial lung diseases (ILDs) caused by mutations mainly involving "telomere-related genes" and "surfactant-related genes". Although, in 2023, European Respiratory Society proposed a statement for FPFs management, these still remain a burden. Our work aimed to evaluate the management and impact of FPF in three Italian different medical settings: University Hospitals (UHs), non-University Hospitals (N-UHs) and outpatient clinics.
methodsThis survey was created by ILDs Study Group Società Italiana di Pneumologia/ Italian Respiratory Society (SIP-IRS) and diffused via email to all SIP-IRS members. The descriptive statistical analysis was conducted through GraphPad Prism software (version 8.0). Results: Twenty participants replied to the survey, of which 65% (13/20) worked at UH while the remaining 25% (6/20) and 5% (1/20) worked at N-UH and outpatient clinics, respectively. Centers with, at least, 150 ILD patients visits/year followed a higher number of FPF patients, regardless of University affiliation (p=0.0046). Despite significant discrepancies in genetic testing and availability of counselling were registered, no statistically significant differences in patients' anamnesis assessment were observed between UHs and N-UHs (p=0.4192 and p=0.6525). However, there were relevant differences in the number of FPF patients undergoing genetic assessment in the Centers with Genetics Lab or Unit inside the Hospital (p=0.0253). There was no consensus regarding the impact of FPF diagnosis on lung transplantation and screening of asymptomatic relatives. Similarly, no differences were reported in antifibrotic prescriptions between UHs and N-UHs. Although the typical UIP pattern was the most common radiological pattern observed in FPF patients, there were no differences in the prevalence of histopathological patterns between UH and N-UH.
conclusionsImproving pulmonologists' knowledge of the approach, diagnosis and management of FPF is a global medical topic. Scientific societies can provide significant support in raising physicians' awareness of this issue.
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