PO54 - Progressive Fibrosing ILD in Portugal: Interim Epidemiological and Clinical Results from a National Multicenter Registry
Sofia Neves (Portugal)1; Alexandra Borba (Portugal)2; Ana Loureiro (Portugal)3; Ana Verónica Cardoso (Portugal)4; Carla António (Portugal)5; Cláudia Guerreiro  (Portugal)6; Catarina Pereira (Portugal)7; David Barros Coelho (Portugal)8; Daniela Neves (Portugal)7; Lígia Fernandes (Portugal)9; Marisa Anciães (Portugal)10; Miguel Lopes (Portugal)11; Nelson Marçal (Portugal)12; Pedro Gonçalo Ferreira (Portugal)13; Rita Pinto Basto (Portugal)14; Rita Simões (Portugal)7; Susana Clemente (Portugal)15; António Morais (Portugal)8 16;
1 - Pulmonology Department, ULS Gaia/Espinho - Vila Nova de Gaia (Portugal); 2 - Pulmonology Department, ULS S José - Hospital de Santa Marta (Portugal); 3 - Pulmonology Department, ULS de Trás-os-Montes e Alto Douro - Vila Real (Portugal); 4 - Pulmonology Department, ULS do Tâmega e Sousa - Penafiel (Portugal); 5 - Pulmonology Department, ULS Viseu Dão-Lafões - Viseu (Portugal); 6 - Pulmonology Department, ULS Algarve - Hospital de Faro (Portugal); 7 - Boehringer Ingelheim - Lisboa (Portugal); 8 - Pulmonology Department, ULS São João - Porto (Portugal); 9 - Pulmonology Department, ULS Arrábida - Setúbal (Portugal); 10 - Pulmonology Department, ULS Amadora/Sintra - Lisboa (Portugal); 11 - Pulmonology Department, ULS Almada-Seixal (Portugal);; 12 - Pulmonology Department, ULS Estuário do Tejo - Vila Franca de Xira (Portugal); 13 - Pulmonology Department - Coimbra University Hospital (Portugal); 14 - Pulmonology Department, ULS Santa Maria - Lisboa (Portugal); 15 - Pulmonology Department, ULS Loures- Odivelas - Lisboa (Portugal); 16 - Faculty of Medicine - University of Porto - Porto (Portugal);
Keywords: Progressive fibrosing interstitial lung disease (PF-ILD); Epidemiology; National registry;
Select the theme: Progressive Pulmonary Fibrosis
Type: Original Papers
Presentation: Poster Presentation

Introduction: Epidemiological and clinical data of progressive fibrosing interstitial lung disease (PF-ILD) in Portugal are scarce. Therefore, a nationwide registry has been established to collect real-world data.

Objectives: Characterize Portugal's PF-ILD epidemiology and patient profile.

Materials and Methods: This national, non-interventional, multicenter study collected data from adult PF-ILD patients across 14 specialized Portuguese hospitals, using ATS/ERS/JTS/ALAT 2018 and INBUILD criteria.

Results: Between January 27, 2023, and February 28, 2025, a total of 1,270 PF-ILD patients were identified (726 enrolled in the study, 527 from aggregated database, and 17 from counter logs), corresponding to a prevalence of 26.3 cases per 100,000 persons (95% CI: 24.6–28.0) and an incidence of 6.5 per 100,000 person-years (95% CI: 5.9–7.1). Most patients were male (67.4%) with a mean (SD) age of 73.7 (10.0) years. The most frequent diagnosis was IPF (n=382, 53.4%), followed by HP (n=159, 22.2%), which was the most predominant non‑IPF PF‑ILD subtype (figure 1). Family history of ILD was reported in 8.8% (n=63) of the patients. Environmental exposures were reported by 58.0% (n=413), primarily to birds (68.7%; n=281), followed by humidity/mold (18.6%, n=77). Common comorbidities included arterial hypertension (56.2%; n=400) and diabetes (24.4%; n=174). Over 90% of patients were former and never-smokers (91.4%; n=381).

At baseline, IPF patients presented relatively preserved FVC (mean FVC 85.5%, SD 19.9) and DLCO of 50.8% (SD 17.8), compared to HP (mean FVC 64.6%, SD 18) or uIIP (mean DLCO of 43.3%, SD 18.1).

Conclusions: This interim analysis provides epidemiological and clinical characterization of PF-ILD patients in Portugal, revealing a significant health burden, with patients often experiencing multiple comorbidities, older age at diagnosis and baseline heterogeneity in lung function across ILD subtypes. Environmental and occupational exposures are common and should be routinely assessed. These results highlight the need for ongoing research and targeted management approaches to address the complexity of PF-ILD in clinical practice.

3364_0.png