The Management of Familial Pulmonary Fibrosis in Different Medical Settings: Where Does That Leave Us? An Italian Nationwide Survey

Overview

Journal Sarcoidosis Vasc Diffuse Lung Dis

Specialties Allergy & Immunology
Cardiology & Vascular Diseases
Pulmonary Medicine

Date 2024 Sep 24

PMID 39315977

Authors

Giorgio Monteleone

ILDs Study Group SipIrs

Laura Bergantini

Miriana DAlessandro

Tommaso Pianigiani

Jacopo Simonetti

Bruno Iovene

Francesco Varone

Giacomo Sgalla

Luca Richeldi

Elena Bargagli

Paolo Cameli

Affiliations

Soon will be listed here.

Abstract

Background And Aim: Familial 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.

Methods: This 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.

Conclusions: Improving 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.

Citing Articles

Polymorphism in Patients with Idiopathic Pulmonary Fibrosis-Does It Really Matter?.

Lewandowska K, Lechowicz U, Rozy A, Falis M, Blasinska K, Jakubowska L Int J Mol Sci. 2025; 26(5).

PMID: 40076835 PMC: 11900561. DOI: 10.3390/ijms26052218.

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