PO66 - Determinants of Disease Progression in Immunosuppressive Therapy–Treated Rare Cystic and Interstitial Lung Diseases: A Multicenter Real-World Study from the TRILD Registry
Ramazan Eren (Turkey)1; Goksel Altinisik Ergur (Turkey)2; Ozlem Ozdemir Kumbasar (Turkey)3; Burcu Baran (Turkey)4; Aslı Alkan (Turkey)5; Gulsah Gunluoglu (Turkey)6; Serap Argun Baris (Turkey)7; Oguz Uzun (Turkey)8; Gulfer Okumus (Turkey)9; Zuleyha Bingol (Turkey)9; Elif Yelda Ozgun Niksarlioglu (Turkey)6; Hulya Dirol (Turkey)10; Inci Gulmez (Turkey)4; Habibe Hezer (Turkey)11; Aylin Ozgen Alpaydin (Turkey)12; Mehmet Parlak (Turkey)10; Aycan Yuksel (Turkey)13; Sureyya Cetin Yilmaz (Turkey)14; Oguz Karcioglu (Turkey)5; Senay Yilmaz (Turkey)15; Pinar Yildiz Gulhan (Turkey)16; Ilknur Basyigit (Turkey)7; Ismail Hanta (Turkey)17; Gokhan Altan (Turkey)9; Ozge Oral Tapan (Turkey)18; Omer Ozbudak (Turkey)10; Kubra Gungor (Turkey)11; Nurdan Kokturk (Turkey)19; Ahmet Ursavas (Turkey)20; Derya Kocakaya (Turkey)21; Nurevsan Kusdogan (Turkey)22; Talat Kilic (Turkey)23; Fatma Arslan (Turkey)3; Nesrin Ocal (Turkey)24; Ilker Yılmam (Turkey)25; Ersin Alkilinc (Turkey)26; Meltem Agca (Turkey)27; Ilgim Vardaloglu (Turkey)28; Emine Arguder (Turkey)11; Isil Uzel (Turkey)29; Baris Demirkol (Turkey)30; Nazli Cetin (Turkey)31; Orkun Eray Terzi (Turkey)32; Ceren Ilgar Akelma (Turkey)1; Ceren Degirmenci (Turkey)18; Aysu Sinem Koc (Turkey)33; Serhat Erol (Turkey)3; Eylem Sercan Ozgur (Turkey)34; Gulru Polat (Turkey)35;
1 - Eskisehir City Hospital, Department of Chest Diseases; 2 - Pamukkale University Faculty of Medicine, Department of Chest Diseases; 3 - Ankara University Faculty of Medicine, Department of Chest Diseases; 4 - Erciyes University Faculty of Medicine, Department of Chest Diseases; 5 - Hacettepe University Faculty of Medicine, Department of Chest Diseases; 6 - Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, Department of Chest Diseases; 7 - Kocaeli University Faculty of Medicine, Department of Chest Diseases; 8 - Ondokuz Mayıs University Faculty of Medicine, Department of Chest Diseases; 9 - Istanbul University Faculty of Medicine, Department of Chest Diseases; 10 - Akdeniz University Faculty of Medicine, Department of Chest Diseases; 11 - Ankara Bilkent City Hospital, Department of Chest Diseases; 12 - Dokuz Eylül University Faculty of Medicine, Department of Chest Diseases; 13 - TOBB University of Economics and Technology Faculty of Medicine, Department of Chest Diseases; 14 - Dicle University Faculty of Medicine, Department of Chest Diseases; 15 - Eskisehir Osmangazi University Faculty of Medicine, Department of Chest Diseases; 16 - Duzce University Faculty of Medicine, Department of Chest Diseases; 17 - Cukurova University Faculty of Medicine, Department of Chest Diseases; 18 - Mugla Sıtkı Kocman University Training and Research Hospital, Department of Chest Diseases; 19 - Gazi University Faculty of Medicine, Department of Chest Diseases; 20 - Uludag University Faculty of Medicine, Department of Chest Diseases; 21 - Marmara University Faculty of Medicine, Department of Chest Diseases; 22 - Gaziantep City Hospital, Department of Thoracic Surgery; 23 - Malatya Inonu University Faculty of Medicine, Department of Chest Diseases; 24 - Health Sciences University, Gulhane Faculty of Medicine, Department of Chest Diseases; 25 - Trakya University Faculty of Medicine, Department of Chest Diseases; 26 - Kocaeli City Hospital, Department of Chest Diseases; 27 - Sureyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital, Department of Chest Diseases; 28 - Bagcılar Training and Research Hospital, Department of Chest Diseases; 29 - Koc University Faculty of Medicine, Department of Chest Diseases; 30 - Basaksehir Cam and Sakura City Hospital, Department of Chest Diseases; 31 - Afyonkarahisar State Hospital, Department of Chest Diseases; 32 - Bursa Yuksek Ihtisas Training and Research Hospital, Department of Chest Diseases; 33 - Istinye University Faculty of Medicine, Department of Chest Diseases; 34 - Mersin University Faculty of Medicine, Department of Chest Diseases; 35 - Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, Department of Chest Diseases;
Keywords: Orphan diseases; Treatment - Anti-inflammatory; Epidemiology;
Select the theme: Rare diseases
Type: Original Papers
Presentation: Poster Presentation
Introduction: Rare cystic and interstitial lung diseases comprise a heterogeneous group of disorders with overlapping clinical and radiological features but distinct pathophysiological mechanisms and clinical courses. Limited real-world data and diagnostic challenges hinder optimal disease management. We aimed to evaluate disease progression and identify predictors of adverse clinical outcomes in a nationwide multicenter cohort of patients with rare cystic and interstitial lung diseases receiving immunosuppressive therapy.
Objectives: This study aimed to evaluate disease progression and its determinants in a nationwide multicenter cohort of patients with rare cystic and interstitial lung diseases receiving immunosuppressive therapy.
Materials and Methods: This multicenter observational study within the Turkish Registry of Rare Interstitial Lung Diseases (TRILD) retrospectively analyzed demographic, clinical, functional, and radiological data of patients with rare cystic and interstitial lung diseases under immunosuppressive therapy.
Results: A total of 106 patients were included (median age 38.5 years [IQR 29.0–48.8]; 64.2% female). PLCH was the most frequent diagnosis (38.7%), followed by LAM (22.6%) and PAP (12.3%). Treatment patterns varied across disease subtypes, with corticosteroids predominantly used in PLCH, PAP, and PPFE, whereas sirolimus was the main therapy in LAM. Despite immunosuppressive therapy, patients with disease progression had significantly lower DLCO, more frequent exercise-induced desaturation, and higher mortality. No significant differences were observed regarding age, sex, smoking status, comorbidities, or FVC. In multivariable logistic regression, lower DLCO independently predicted disease progression (OR 0.97, 95% CI 0.95–0.99; p=0.026). ROC analysis demonstrated a moderate discriminative performance of DLCO for predicting progression (AUC=0.70), with an optimal cutoff value of 43.5%. Male sex showed a trend toward increased progression risk but did not reach statistical significance.
Conclusions: Reduced DLCO was independently associated with disease progression in patients with rare cystic lung diseases receiving immunosuppressive therapy and should be considered a key functional marker for risk stratification and follow-up.