Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Greece: results from the Greek idiopathic pulmonary fibrosis registry (INDULGE IPF)

Background Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterised by progressive fibrosis associated with irreversible lung function decline, respiratory failure and high mortality. The establishment of national IPF registries worldwide has provided valuable “real-world” data on IPF patients outside clinical trials, facilitating better characterisation and management of the disease. Methods Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF) was a national, multicentre, prospective registry assessing the characteristics and management of IPF patients under “real-world” clinical conditions in Greece. Results Between April 5, 2017, and April 2, 2019, a total of 301 patients (mean age: 71.6 years; 81.7% male; 71.1% current or former smokers) were enrolled across seven pulmonary clinics in Greece. Among these patients, 96 (32%) were newly diagnosed within the past 6 months (“incident IPF”), whereas 205 (68%) had a disease duration of ≥ 6 months (“prevalent IPF”), with a median time since initial diagnosis of 10.4 months (range: 0–233.3) for all patients. The diagnosis was made according to the 2011 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association (ATS/ERS/JRS/ALAT) guidelines, with the majority of patients (79.5%) demonstrating a definite usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and only 8% undergoing surgical lung biopsy. At the time of enrolment, mean ± standard deviation (SD) forced vital capacity (FVC) was 78.2 ± 20.7% predicted and the mean ± SD diffusing capacity of the lung for carbon monoxide (DL CO ) was 48.2 ± 17.8% predicted. Most patients were treated with one of the two currently approved antifibrotics, nintedanib and pirfenidone, administered to 41.9% and 39.9% of patients, respectively. Conclusions The INDULGE IPF registry provides insights into the characteristics and natural course of IPF patients, as well as their routine clinical management in Greece. Trial registration The study was registered at ClinicalTrials.gov under the identifier NCT03074149 on January 4, 2017.