Patients with Idiophatic Pulmonary Fibrosis (IPF), serving as a prototype of a progressive fibroproliferative disorder.

Patients with non-IPF interstitial lung diseases, presenting a progressive fibrosing phenotype, or acute exacerbations.

Patients with fibrotic non-IPF interstitial lung diseases that are stable during a minimum follow-up of 24 months.

To establish the first Portuguese registry and biobank of PF-ILDs, comprising both extensive patient-level data, and systematic biological sampling (DNA, RNA, plasma, serum, bronchoalveolar lavage, lung tissue) at baseline and repeated biological sampling of blood and pharyngeal swabs performed at 6, 12 and 18 months, or whenever progression criteria are met or an acute exacerbation occurs. Participants will have regular visits at maximum intervals of 6 months, when their clinical condition and lung function tests are reassessed. A high resolution computed tomography (HRCT) scan of the lung will be performed every 12 months. Progressive fibrosis will be diagnosed based on meeting at least two of the following three criteria, occurring within the last year: (i) worsening of symptoms; (ii) absolute decline in FVC ≥5% predicted or absolute decline in DLCO (corrected for Hb) ≥10% predicted; (iii) increased extent of fibrotic changes on HRCT.

Characterization of blood and tissue transcriptional signatures of progression and acute exacerbations, and validate findings at the protein expression level, which could be easily converted for clinical use as biomarkers.

To assess the impact of microbiome features in clinical progression and higher risk of acute exacerbation