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  9. Predictive And Prognostic Markers
  11. Outcomes And Predictors Of Progression In Progressive Pulmonary Fibrosis

Outcomes and predictors of progression in progressive pulmonary fibrosis

Zekai Cen1, Tiantian Cen1, Qunli Ding1

  • 1Department of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang, China.

Annals of Medicine
|September 23, 2024

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View abstract on PubMed

Summary
This summary is machine-generated.

Older age, high tumor markers, and poor lung function predict progressive pulmonary fibrosis (PPF) development. Increased pulmonary artery pressure and acute exacerbations are key risk factors for PPF patient mortality.

Area of Science:

  • Pulmonology
  • Interstitial Lung Diseases
  • Fibrotic Interstitial Lung Diseases

Background:

  • Progressive pulmonary fibrosis (PPF) is a severe interstitial lung disease (ILD) subset characterized by a progressive fibrosing (PF) phenotype.
  • Patients with PPF experience significant declines in lung function, exercise capacity, and overall quality of life.
  • Understanding the clinical characteristics and progression factors of PPF is crucial for improving patient outcomes.

Purpose of the Study:

  • To investigate the clinical characteristics of patients with a progressive fibrosing (PF) phenotype of interstitial lung disease (ILD).
  • To identify potential factors associated with disease progression from fibrotic ILD (f-ILD) to PPF.
  • To analyze the survival outcomes of patients diagnosed with PPF.

Main Methods:

Keywords:
Progressive pulmonary fibrosisacute exacerbationinterstitial lung diseasetumour markers

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  • Retrospective review of ILD patient data from January 2011 to December 2022 at The First Affiliated Hospital of Ningbo University.
  • Identification of patients with a PF phenotype based on established PPF clinical practice guidelines.
  • Analysis of baseline clinical information, laboratory parameters, and imaging findings (chest HRCT).

    • Main Results:

    • Patients with PPF exhibited higher tumor marker levels and reduced pulmonary function (lower FVC, DLCO) at baseline compared to non-PPF patients.
    • Multivariate logistic regression identified age >65, elevated LDH, high CA-153, reduced FVC/DLCO, and UIP-like HRCT patterns as predictors of PPF progression.
    • The PPF group showed significantly poorer survival rates; elevated SPAP (≥ 37 mmHg) and acute exacerbations were independent prognostic factors for mortality.

    Conclusions:

    • Older age, elevated tumor markers (CA-153, LDH), impaired pulmonary function, and specific HRCT patterns are associated with f-ILD progression to PPF.
    • Increased systolic pulmonary artery pressure (SPAP) and acute exacerbations (AE) are significant independent risk factors for mortality in PPF patients.
    • Close monitoring and targeted interventions for patients with these risk factors are essential for improving the prognosis of PPF.