Overview
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.
Eligibility
Inclusion Criteria:
- Age over 18 years
- IPF of any degree of severity, diagnosed according to the 2022 ATS/ERS/JRS/ALAT guidelines
Exclusion Criteria:
- Refusal to participate in the study
- Patients unable to provide informed consent for participation in the study
- IPF exacerbation in the 6 months prior to enrollment
- Previous diagnosis of chronic airway disease (e.g., bronchial asthma, chronic obstructive bronchitis, bronchiectasis with a cause other than IPF)
- Presence of bronchial obstruction defined by an FEV1/FVC (or FEV1/VC) ratio below the lower limit of normal
- Chronic therapy with long-acting bronchodilators or combinations of bronchodilators and inhaled corticosteroids
- Inability of the patient to perform reproducible pulmonary function tests
- Chronic treatment with systemic corticosteroids or immunosuppressants
- Concomitant lung or pleural cancer
- Pregnancy or breastfeeding women