Abstract

Background: Idiopathic Pulmonary Fibrosis (IPF) is a rare disease associated with a median survival time of 2 to 5 years after diagnosis. Data on natural course of IPF in Italy are scarce. Aims and objectives: We reported the interim description of IPF symptom course during 3 months in a sample of IPF-diagnosed Italian patients who will be followed for 1 year. Methods: FIBRONET is an observational, multicentre, prospective cohort study on ≥40 years-old Italian IPF newly-diagnosed patients. Evaluations at 3, 6, 9, 12 months from baseline are planned. We assessed IPF symptoms’ presence at study visits according to clinical judgment and dyspnea at baseline by means of the Modified Medical Research Council dyspnea scale score ≥2. Results: Data collection is ongoing; preliminary baseline data on 168 patients [mean±SD age: 69.5 (±7.4) years, 146 (87%) males] were described. At baseline 48 (43%) patients showed significant dyspnea and 19 (11%) were under IPF pharmacological therapy. Considering 116 patients who performed 3-month follow up visit, 105 (91%) and 71 (61%) had IPF symptoms at baseline and after 3 months respectively. Cough and fatigue were the most frequently reported symptoms at baseline [66 (57%) and 64 (55%) patients respectively] and at 3-month follow-up visit [43 (37%) and 43 (37%) patients respectively]. Considering 105 patients with symptoms at baseline, 36 (34%) had no symptoms at all after 3 months. Surprisingly, after 3-months of follow up only 65 (56%) patients had ongoing IPF pharmacological therapy. Conclusions: Data gathered through the FIBRONET study will provide insights on clinical course of IPF in Italian patients treated under real-world conditions.

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