PO55 - Diagnostic Journey and Care Pathways in Progressive Fibrosing ILDs in Portugal: interim results from a national registry
Cláudia Guerreiro  (Portugal)1; Alexandra Borba (Portugal)2; Ana Loureiro (Portugal)3; Ana Verónica Cardoso (Portugal)4; Carla António (Portugal)5; Catarina Pereira (Portugal)6; David Barros Coelho (Portugal)7; Daniela Neves (Portugal)6; Lígia Fernandes (Portugal)8; Marisa Anciães (Portugal)9; Miguel Lopes (Portugal)10; Nelson Marçal (Portugal)11; Pedro Gonçalo Ferreira (Portugal)12; Rita Pinto Basto (Portugal)13; Rita Simões (Portugal)6; Susana Clemente (Portugal)14; Sofia Neves (Portugal)15; António Morais (Portugal)16 17; Vanda Areias (Portugal)18;
1 - Pulmonology Department, ULS Algarve - Hospital de Faro (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 - Boehringer Ingelheim - Lisboa (Portugal); 7 - Pulmonology Department, ULS São João - Porto (Portugal); 8 - Pulmonology Department, ULS Arrábida - Setúbal (Portugal); 9 - Pulmonology Department, ULS Amadora/Sintra - Lisboa (Portugal); 10 - Pulmonology Department, ULS Almada-Seixal - Faro (Portugal); 11 - Pulmonology Department, ULS Estuário do Tejo - Vila Franca de Xira (Portugal); 12 - Pulmonology Department - Coimbra University Hospital (Portugal); 13 - Pulmonology Department, ULS Santa Maria - Lisboa (Portugal); 14 - Pulmonology Department, ULS Loures-Odivelas - Lisboa (Portugal); 15 - Pulmonology Department, ULS Gaia/Espinho - Vila Nova de Gaia (Portugal); 16 - Pulmonology Department, ULS São João (Portugal); 17 - Faculty of Medicine - University of Porto - Porto (Portugal); 18 - Serviço de Pneumologia, ULS Algarve;
Keywords: Progressive fibrosing interstitial lung diseases (PF-ILD); Diagnostic pathways; National registry;
Select the theme: Progressive Pulmonary Fibrosis
Type: Original Papers
Presentation: Poster Presentation

Introduction: There is currently a lack of understanding of the disease management and care pathways of PF-ILD patients in the Portuguese healthcare setting.

Objectives: Characterize Portugal's PF-ILD diagnostic pathways and management.

Materials and Methods: This national, non-interventional, multicenter study collected real-world data from adult PF-ILD patients across 14 specialized Portuguese hospitals based on IPF (ATS/ERS/JTS/ALAT 2018) and INBUILD criteria. Data collection included medical history, demographics, diagnosis, treatments and adverse events (AEs).

Results: Between January 27, 2023, and February 28, 2025, 1270 PF-ILD patients were identified (726 enrolled in the study, 527 from an aggregated database, and 17 from counter logs). The most frequent diagnosis were IPF (53.4%; n=382), hypersensitivity pneumonitis (22.2%; n=159) and autoimmune ILDs (10,2%; n=73).

At diagnosis, the most frequent presenting symptoms were dyspnea (76.0%; n=544), cough (65.2%; n=467) and fatigue (34.1%; n=244). Notably, 18 patients (2.5%) were asymptomatic. Most patients (89,8%; n=643) were diagnosed through MDT discussions, with referrals predominantly originating from public sector (83.9%, n=579), particularly from GPs (32.2%; n=222) and respiratory specialists (28.3%; n=195). The mean (SD) time from symptom onset to referral was 26.1 (41.5) months and 34.8 (43.3) months to diagnosis.

Antifibrotic therapy was prescribed in 80.7% of cases (n=578), predominantly nintedanib (74.9%; n=433). IPF shows the highest antifibrotic usage (84.3%, n=322), followed by uIIP (79.4%, n=27), HP (76.1%, n=121) and CTD-ILD (75.3%, n=55). A total of 58 AEs were reported, mostly mild or moderate.

Conclusions: This interim analysis exposes major delays in the PF‑ILD diagnostic pathway in Portugal, with nearly three years from symptom onset to diagnosis, underscoring the need for greater clinical awareness and faster referral. The dominant role of public‑sector referrals, namely GPs are vital in the diagnostic pathway with opportunities for improvement. The strong contribution of MDT discussions supports robust diagnostic practice. Extended longitudinal follow-up is necessary to better understand disease progression and outcomes, which may be underreported.

3276_0.png