PO64 - The Diagnostic Journey in Rare Interstitial Lung Diseases in Türkiye: A Multicenter TRILD Registry Study on Healthcare Levels, Referral Pathways, and Timing
Ceren Degirmenci (Turkey)1; Nazli Cetin (Turkey)2; Orkun Eray Terzi (Turkey)3; Goksel Altinisik (Turkey)4; Ozlem Ozdemir Kumbasar (Turkey)5; Ilknur Basyigit (Turkey)6; Sureyya Cetin Yilmaz (Turkey)7; Burcu Baran (Turkey)8; Asli Alkan (Turkey)9; Kubra Gungor (Turkey)10; Zuleyha Bingol (Turkey)11; Gulfer Okumus (Turkey)11; Gokhan Altan (Turkey)11; Serhat Erol (Turkey)5; Habibe Hezer (Portugal)10; Serap Argun Baris (Turkey)6; Oguz Karcioglu (Turkey)9; Inci Gulmez (Turkey)8; Pinar Yildiz Gulhan (Turkey)12; Damla Serce Unat (Turkey)13; Celalettin Korkmaz (Turkey)14; Omer Ozbudak (Turkey)15; Nurdan Kokturk (Turkey)16; Talat Kilinc (Turkey)17; Gulsah Gunluoglu (Turkey)18; Aliye Gamze Calis (Turkey)15; Hulya Dirol (Turkey)15; Mehmet Parlak (Turkey)15; Kubra Taskaraca Karabacak (Turkey)16; Ilker Yilmam (Turkey)19; Senay Yilmaz (Turkey)20; Elif Yelda Niksarlioglu (Turkey)21; Elif Yildirim (Turkey)18; Nesrin Ocal (Turkey)22; Oguz Uzun (Turkey)23; Fatma Arslan (Turkey)5; Aylin Ozgen Alpaydin (Turkey)24; Ramazan Eren (Turkey)18; Emine Aksoy (Turkey)21; Ezgi Demirdogen (Turkey)25; Sermin Borekci (Turkey)26; Nazli Huma Teke (Turkey)27; Derya Kocakaya (Turkey)28; Gulru Polat (Turkey)29; Aycan Yuksel (Turkey)30; Ceren Ilgar Akelma (Turkey)31; Ilgim Vardaloglu (Turkey)32; Aysu Sinem Koc (Turkey)33; TRILD Study Group (Turkey)34; Eylem Sercan Ozgur (Turkey)35;
1 - Mugla Training and Research Hospital, Department of Chest Diseases; 2 - Afyonkarahisar State Hospital, Department of Chest Diseases; 3 - Bursa Yuksek Ihtisas Training and Research Hospital, Department of Chest Diseases; 4 - Pamukkale University Faculty of Medicine, Department of Chest Diseases; 5 - Ankara University Faculty of Medicine, Department of Chest Diseases; 6 - Kocaeli University Faculty of Medicine, Department of Chest Diseases; 7 - Dicle University Faculty of Medicine, Department of Chest Disease; 8 - Erciyes University Faculty of Medicine, Department of Chest Diseases; 9 - Hacettepe University Faculty of Medicine, Department of Chest Diseases; 10 - Ankara Bilkent City Hospital, Department of Chest Diseases; 11 - Istanbul University Faculty of Medicine, Department of Chest Diseases; 12 - Duzce University Faculty of Medicine, Department of Chest Diseases; 13 - Izmir Bakircay University Faculty of Medicine, Department of Chest Diseases; 14 - Necmettin Erbakan University Faculty of Medicine, Department of Chest Diseases; 15 - Akdeniz University Faculty of Medicine, Department of Chest Diseases; 16 - Gazi University Faculty of Medicine, Department of Chest Diseases; 17 - Malatya Inonu University Faculty of Medicine, Department of Chest Diseases; 18 - Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, Department of Chest Diseases; 19 - Trakya University Faculty of Medicine, Department of Chest Diseases; 20 - Eskisehir Osmangazi University Faculty of Medicine, Department of Chest Diseases; 21 - Sureyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital, Department of Chest Diseases; 22 - Health Sciences University, Gulhane Faculty of Medicine, Department of Chest Diseases; 23 - Ondokuz Mayıs University Faculty of Medicine, Department of Chest Diseases; 24 - Dokuz Eylül University Faculty of Medicine, Department of Chest Diseases; 25 - Uludag University Faculty of Medicine, Department of Chest Diseases; 26 - Istanbul University–Cerrahpaşa Faculty of Medicine, Department of Chest Diseases; 27 - Basaksehir Cam and Sakura City Hospital, Department of Chest Diseases; 28 - Marmara University Faculty of Medicine, Department of Chest Diseases; 29 - Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, Department of Chest Diseases; 30 - TOBB University of Economics and Technology Faculty of Medicine, Department of Chest Diseases; 31 - Eskisehir City Hospital, Department of Chest Diseases; 32 - Bagcilar Training and Research Hospital, Department of Chest Diseases; 33 - Istinye University Faculty of Medicine, Department of Chest Diseases; 34 - Turkish Thoracic Society; 35 - Mersin University Faculty of Medicine, Department of Chest Diseases;
Keywords: Rare interstitial lung diseases; diagnostic delay; diagnostic odyssey;
Select the theme: Rare diseases
Type: Original Papers
Presentation: Poster Presentation
Introduction: Rare interstitial lung diseases often involve diagnostic delays due to their low prevalence and nonspecific presentation. Patients with rare interstitial lung diseases often undergo a prolonged diagnostic odyssey before receiving an accurate diagnosis.
Objectives: Rare interstitial lung diseases often involve diagnostic delays due to their low prevalence and nonspecific presentation. The resulting diagnostic odyssey may delay referral to specialized centers and timely initiation of appropriate management.
Materials and Methods: Patients with rare interstitial lung disease followed in pulmonology clinics across Türkiye were included. Demographics, symptom onset, initial healthcare contact and level, timing of pulmonology referral, HRCT, diagnostic method, and diagnosis date were analyzed.
Results: A total of 587 patients from 44 participating centers were included; 53% were female, with a mean age of 45.3 years. Only 29.7% were diagnosed at the first center, while 15.1% visited three or more centers. Median times from symptom onset were 33 days to first physician, 50 days to pulmonology consultation, and 219.5 days to diagnosis. Diagnostic delay was longest in pleuroparenchymal fibroelastosis and shortest in pulmonary alveolar microlithiasis and pulmonary alveolar proteinosis (p<0.05). Patients with longer diagnostic delays had a significantly higher prevalence of comorbidities (p=0.014). Initial presentation to the emergency department was not associated with a shorter time to diagnosis (p=0.289).
Conclusions: This nationwide TRILD registry study shows marked diagnostic delays in rare interstitial lung diseases in Türkiye, mainly before pulmonology referral, underscoring the need for better awareness and referral pathways. In addition, patients with longer diagnostic delays had a higher prevalence of comorbidities, suggesting that coexisting conditions may further complicate the recognition of these rare diseases. Furthermore, increasing the availability of educational programs on these diseases is essential to enhance awareness among healthcare professionals and support earlier diagnosis and appropriate referral pathways.