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Background <p>Idiopathic 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.</p> Methods <p>A 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.</p> Results <p>A 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 (<i>p</i> &lt; 0.0001). The average annual incidence was 4.6 cases per 100,000 inhabitants, with significantly higher incident cases among males (<i>p</i> &lt; 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, <i>p</i> &lt; 0.0001) and with age at diagnosis (adjHR = 1.04, 95%CI: 1.03–1.05, <i>p</i> &lt; 0.0001).</p> Conclusions <p>The 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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月19日

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  • Gorini Francesca,
  • Santoro Michele,
  • Pierini Anna,
  • Cameli Paolo,
  • Chimera Davide,
  • Lavorini Federico,
  • Pistelli Francesco,
  • Rosi Elisabetta,
  • Tavanti Laura,
  • Tomassetti Sara,
  • Laura Carrozzi,
  • Bargagli Elena,
  • Coi Alessio

摘要

Background

Idiopathic 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.

Methods

A 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.

Results

A 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).

Conclusions

The 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.