Pulmonary Alveolar Proteinosis: a 3-country cohort study Greece, Türkiye, Cyprus
Bildiriler > Özet Bildiri
Yazarlar: Spyros A Papiris, Efsun Gonca Uğur Chousein, Maria Kallieri, Nesrin MOĞULKOÇ BISHOP, Andriana I Papaioannou, Frangiskos Frangopoulos, Tonia Adamide, Lykourgos Kolilekas, Burak Gökçe, Nesrin Ocal, Despoina Papakosta, Berna Akıncı Özyürek, Vasilios Tzilas, Enver Yalnız, Georgia Kontonasiou, Ezgi Demirdöğen, Emmanouil Verykakis, Ahmet Ursavaş, Elvira-Markella Antonogiannaki, Pelin Pınar Deniz, Vasiliki Apollonatou, Ismail Hanta, Dimitrios Chatzis, Anastasia Levounets, Nicoletta Olympiou, Ilias Dimeas, Zoe Daniil, Nikolaos Roussakis, Andrea Schams, Delphine Monnier, Christina Rampiadou, Paschalis Steiropoulos, Katerina Markopoulou, Nikolaos Tzanakis, Konstantinos Gourgoulianis, Demosthenes Bouros, Brenna Carey, Bruce Trapnell, Matthias Griese, Erdoğan Çetinkaya, Effrosyni D. Manali
Rare ILD/DPLD, , , Uluslararası, Özet Bildiri, ss PA3028-PA3028
DOI: 10.1183/13993003.congress-2025.pa3028
Özet
Background: Pulmonary Alveolar Proteinosis (PAP) is a rare disease with inappropriate accumulation of surfactant in alveoli. Aim to characterize 3 cohorts in 3 Mediterranean neighbors (Greece, Türkiye, Cyprus) the last 20 years. Methods: Epidemiological, clinical and functional data were analyzed Results: Included are 124 patients: 32% Greece, 65% Türkiye, 3% Cyprus; 109 (88%) autoimmune PAP (aPAP), 13 (11%) secondary and 1 (1%) congenital; 49% male, 65% ever-smokers, followed-up 39 (16-83) months. At diagnosis median (IQR) age 39 (30-47) years, %-predicted FVC 77 (64-91), DLCO 55 (37-68),14% in LTOT. 7% had died. Whole lung lavage (WLL) in 51%, history of inhaled-GMCSF (sargramostim or molgramostim) in 44%. During FU, patients ameliorated [FVC% (p=0.007), DLCO% (p=0.001)]. Baseline characteristics of the three cohorts were similar. Significant differences were: WLL in Türkiye 64% vs 26% in Greece and Cyprus (p<0.001) and history of i-GMCSF as sole therapy 51% in Greece vs 9% in Türkiye (p<0.001). Deceased patients were more likely to be older (p=0.009), have secondary PAP (p<0.001), more comorbidities (p=0.035), higher oxygen-needs (p<0.001), worse functional status at FU (p=0.001) and complicated by pulmonary fibrosis (p<0.001). Patients who received i-GMCSF as sole therapy had no significant differences regarding age, disease severity or outcome compared to those with a history of WLL alone or in combination with i-GMCSF. Conclusion: In Türkiye WLL was the mainstay of treatment, while in Greece i-GM-CSF. Secondary PAP, age and pulmonary fibrosis negatively influenced prognosis. i-GMCSF as the sole therapy was not inferior to WLL or WLL followed by i-GMCSF irrespective of the severity of patients.