Idiopathic Pulmonary Fibrosis (IPF) is a chronic, fibrosing, and progressive lung disease of unknown cause, whose incidence increases proportionally from the age of 60. It is characterized by a poor prognosis. Antifibrotic therapy can slow the progression of the disease and reduce mortality, but the life expectancy is less than 7-10 years in the vast majority of patients with IPF. There are no studies in the literature that have evaluated the presence of small airway disease in patients with IPF prior to the initiation of pharmacological therapy, using the nitrogen washout test. This test is currently considered the only non-invasive method capable of detecting ventilation inhomogeneity and closing volume, which are indicators of small airway dysfunction. The investigators carried out an Italian prospective, observational, multicenter study with the primary aim to assess the prevalence of small airway disease measured by the nitrogen washout test (evaluating the following functional parameters: phase 3 slope, closing volume, closing capacity, closing volume/vital capacity, closing capacity/total lung capacity, and phase 4 slope) in a group of patients with IPF at the time of diagnosis, before the initiation of antifibrotic therapy. During outpatients visits clinical, functional and radiological data will be collected. Results will be compared to an healthy control group matched with IPF population. Variations in small airways disease parameters will be assessed after one year of antifibrotic treatment.
Study Type
OBSERVATIONAL
Enrollment
100
Pulmonology Unit, ASST Santi Paolo e Carlo. Department of Health Sciences, University of Milan, Milan (Italy)
Milan, Lombardy, Italy
RECRUITINGDivision of Respiratory Diseases, L. Sacco University Hospital, ASST Fatebenefratelli-Sacco, Milan, Italy. Department of Biomedical and Clinical Sciences, Università Degli Studi di Milano, Milano, Italy
Milan, Lombardy, Italy
RECRUITINGPrevalence of small airway disease in a group of patients with IPF at the time of diagnosis, before the initiation of antifibrotic therapy.
To evaluate the percentage of patients with IPF at the time of diagnosis, before the initiation of antifibrotic therapy, with small airway disease measured by the nitrogen washout test (assessing the following functional parameters: phase 3 slope, closing volume, closing capacity, closing volume/vital capacity, closing capacity/total lung capacity, and phase 4 slope).
Time frame: 1 year
Relationship between computed tomography (CT) features and functional parameters indicative of small airway disease.
To evaluate the relationship between CT features (radiologic pattern type, presence/absence of mediastinal lymphadenopathy, presence/absence of any type of emphysema involving ≥5% of total lung volume, extent of disease, disease progression, air trapping, black lung) and functional parameters indicative of small airway disease.
Time frame: 1 year
To compare the indices of small airway dysfunction and the prevalence of small airway disease in patients with IPF and age-matched healthy subjects
To compare the indices of small airway dysfunction and the prevalence of small airway disease in patients with IPF and age-matched healthy subjects (without respiratory comorbidities).
Time frame: 1 year
Relationship between clinical and epidemiological characteristics of the cohort and the presence and severity of small airway disease
To study the relationship between clinical and epidemiological characteristics of the cohort and the presence and severity of small airway disease
Time frame: 1 year
Prevalence of small airway disease in patients with IPF and concomitant emphysema with patients with IPF alone
To compare the prevalence of small airway disease in patients with IPF and concomitant emphysema (combined pulmonary fibrosis and emphysema, CPFE), defined as the presence of any type of emphysema involving ≥5% of total lung volume, with patients with IPF alone.
Time frame: 1 year
Correlation between functional characteristics, parameters of small airway dysfunction and fibrosis extent indices on chest CT-scan
To evaluate the correlation between functional characteristics: forced expiratory flow at 25-75% of vital capacity (FEF25-75), forced vital capacity (FVC) and diffusing of the lung for carbon monoxide (DLCO) with parameters of small airway dysfunction from the Single Breath Nitrogen (SBN2) test, as well as disease extent indices on chest CT-scan
Time frame: 1 year
Relationship between exercise performance and the presence and severity of small airway disease
To evaluate the relationship between exercise performance (meters, oxygen saturation nadir), assessed by the six-minute walk test distance, and the presence and severity of small airway disease
Time frame: 1 year
Relationship between dyspnea degree and the presence and severity of small airway disease.
To evaluate the relationship between the degree of dyspnea, measured by the modified Medical Research Council (mMRC) scale, and the presence and severity of small airway disease.
Time frame: 1 year
Clinical and functional characteristics of IPF patients with different alterations in the DLCO subcomponents
To evaluate the clinical and functional characteristics of IPF patients with different alterations in the DLCO subcomponents (alveolar volume - VA \<80% predicted, Transfer Coefficient of CO - KCO \<80% predicted, ventilation inhomogeneity - VA/Total Lung Capacity (TLC) \<0.8)
Time frame: 1 year
Relationship between different scores as the GAP index, CPI, KBILD and UCSD Shortness of Breath Questionnaire, and the presence and severity of small airway disease
To evaluate the relationship between different scores as the Gender Age Physiology (GAP) index, Composite Physiologic Index (CPI) , the King's Brief Interstitial Lung Disease (KBILD) score and the University of California San Diego (UCSD) Shortness of Breath Questionnaire, and the presence and severity of small airway disease;
Time frame: 1 year
Variation of small airway dysfunction parameters in IPF patients after one year of antifibrotic treatment.
Evaluate presence and variation of small airway dysfunction parameters from the Single Breath Nitrogen (SBN2) test in IPF patients after one year of antifibrotic therapy.
Time frame: 1 year
Prevalence of tidal expiratory flow limitation at the time of diagnosis, before the initiation of antifibrotic therapy and after one year of antifibrotic treatment.
To evaluate the percentage of patients with IPF with tidal expiratory flow limitation measured by means of Negative Expiratory Pressure (NEP), at the time of diagnosis, before the initiation of antifibrotic therapy and after one year of therapy with antifibrotic treatment
Time frame: 1 year
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