Evidence map›Paper›PMID 41555350›Full record

ArticleOrphanet journal of rare diseases2026

Epidemiology of idiopathic pulmonary fibrosis: a population registry-based study.

Gorini Francesca, Santoro Michele, Pierini Anna, Cameli Paolo, Chimera Davide, Lavorini Federico, Pistelli Francesco, Rosi Elisabetta, Tavanti Laura, Tomassetti Sara and 3 more

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In one paragraph

Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. Review
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

13 authors.

Gorini FrancescaUnit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy. fgorini@ifc.cnr.it.
Santoro MicheleUnit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
Pierini AnnaUnit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
Cameli PaoloDepartment of Medicine, Surgery and Neurosciences, Respiratory Diseases Unit, University of Siena, Siena, Italy.
Chimera DavidePulmonary Unit, Cardiothoracic and Vascular Department, Pisa Hospital and University of Pisa, Pisa, Italy.
Lavorini FedericoDepartment of Experimental and Clinical Medicine, Careggi University Hospital and University of Florence, Florence, Italy.
Pistelli FrancescoPulmonary Unit, Cardiothoracic and Vascular Department, Pisa Hospital and University of Pisa, Pisa, Italy.
Rosi ElisabettaPulmonary Unit, Careggi University Hospital, Florence, Italy.
Tavanti LauraPulmonary Unit, Cardiothoracic and Vascular Department, Pisa Hospital and University of Pisa, Pisa, Italy.
Tomassetti SaraDepartment of Experimental and Clinical Medicine, Careggi University Hospital and University of Florence, Florence, Italy.
Laura CarrozziPulmonary Unit, Cardiothoracic and Vascular Department, Pisa Hospital and University of Pisa, Pisa, Italy.
Bargagli ElenaDepartment of Medicine, Surgery and Neurosciences, Respiratory Diseases Unit, University of Siena, Siena, Italy.
Coi AlessioUnit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIdiopathic pulmonary fibrosis (IPF) is a chronic and progressive respiratory rare disease characterized by an irreversible loss of lung function, with unknown etiology and poor prognosis.

methodsA population registry-based study was conducted to provide estimates of prevalence, incidence and survival of IPF. The study included all cases diagnosed with IPF in the years 2000–2022 and residing in Tuscany, Italy. Prevalence as of December 31, 2022, was calculated by sex and age class. Incidence was calculated across the period 2018–2022. Survival at 1, 5 and 10 years from diagnosis with 95% confidence intervals (CI) was estimated by sex and age class using the Kaplan-Meyer method. The independent effect of sex and age at diagnosis on survival was estimated by Cox proportional hazard model.

resultsA total of 1,388 subjects with IPF were diagnosed during the study period. The prevalence as of 31st December 2022 was 21.5 cases (95%CI: 20.0–23.0) per 100,000 inhabitants, with a significantly higher frequency in men and in 70–79 years age group (p < 0.0001). The average annual incidence was 4.6 cases per 100,000 inhabitants, with significantly higher incident cases among males (p < 0.0001). Survival at 1, 5 and 10 years from diagnosis was 91.3%, 51.4% and 22.2%, respectively. Women exhibited a longer survival than men (34.8% vs. 17.3% at 10 years), while patients under 70 had the highest survival rate at 58.2% (95% CI: 52.5–63.4) at five years. Cox regression model confirmed a higher risk of mortality for men (adjusted Hazard Ratio – adjHR = 1.52, 95%CI: 1.25–1.84, p < 0.0001) and with age at diagnosis (adjHR = 1.04, 95%CI: 1.03–1.05, p < 0.0001).

conclusionsThe higher prevalence and incidence rates of IPF among men, as indicated by this population registry-based study, align with recent data reported in Europe. Furthermore, IPF is consistently identified as a disease with a poor prognosis, especially in male patients. Therefore, early and accurate diagnosis, coupled with timely management, is essential to improve the care and treatment outcomes for these patients.

Indexed as

Idiopathic Pulmonary FibrosisAdultAgedAged, 80 and overFemaleHumansIncidenceItalyMaleMiddle AgedPrevalenceRegistriesIdiopathic pulmonary fibrosisIncidencePopulation registry-based studyPrevalenceSurvival

Identifiers

PMID41555350
PMCPMC12903404

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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 OpenQuestion graph.