Pulmonary Alveolar Proteinosis in Greece–Türkiye–Cyprus: Answers in a Real‐Life Comparison
Makaleler > Tam Makale · 2026
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, Despina Papakosta, Berna Akıncı Özyürek, Vasilios Tzilas, Enver Yalnız, Georgia Kontonasiou, Ezgi Demirdöğen, Emmanouil Verykakis, Ahmet Ursavaş, Elvira‐Markela Antonogiannaki, Pelin Pınar Deniz, Vasiliki Apollonatou, Ismail Hanta, Dimitrios Chatzis, Anastasia Levounets, Nicoletta Olympiou, Kalliopi Vandorou, Ilias Dimeas, Zoe Daniil, Nikolaos Roussakis, Andrea Schams, Delphine Monnier, Christina Rampiadou, Paschalis Steiropoulos, Katerina Markopoulou, Nikolaos Tzanakis, Konstantinos Gourgoulianis, Spyridon Prountzos, Demosthenes Bouros, Brenna Carey, Bruce Trapnell, Matthias Griese, Erdoğan Çetinkaya, Effrosyni D. Manali
Respirology, Uluslararası, Wos, 2026
DOI: 10.1002/resp.70288
Özet
ABSTRACT Background and Objective Pulmonary alveolar proteinosis (PAP) is a rare disease of inappropriate alveolar surfactant accumulation. The study objective was to compare Greece–Türkiye–Cyprus cohorts, followed‐up for 20 years. Methods Data were retrieved retrospectively by chart review. Results One hundred and thirty patients, Greece 39, Türkiye 87, Cyprus 4, were included; 115 (89%) autoimmune (a)PAP, 13 (10%) secondary, 1 (1%) hereditary. Abnormal anti‐GM‐CSF antibody titre was available in 86/115 patients, further analyzed for aPAP. Forty (46%) were male, with median (IQR) age at diagnosis of 40 (31–50) years. At median (IQR) duration of clinical follow‐up of 39 (18–78) months, three patients died. Non‐survivors had higher rates of cardiovascular disease [67% vs. 5%, p < 0.001], LTOT (67% vs. 13%, p = 0.001), pulmonary fibrosis [67% vs. 5%] and worse functional status at follow‐up [FVC% pred 49 (29) vs. 87 (74–97); p = 0.026, DLCO% pred 21 (46) vs. 72 (58–82); p = 0.016]. aPAP patients were stratified by therapy group (no WLL‐no i‐GM‐CSF, WLL alone, i‐GMCSF alone and both WLL and iGM‐CSF). WLL was more frequently performed in Türkiye and i‐GMCSF alone in Greece ( p = 0.006). All therapies were effective. No difference was detected on outcome when sole iGM‐CSF was compared to WLL alone or in combination with iGM‐CSF [deceased/alive 0%/100% vs. 7%/93%, p = 0.491]. Sole iGM‐CSF significantly ameliorated clinical and functional parameters of disease severity [DLCO% ( p = 0.013), SatO 2 ( p = 0.002), 6MWT ( p = 0.026)]. Across all therapy groups, pulmonary fibrosis eliminated beneficial response to treatment. Conclusion This first real‐life comparison of aPAP cohorts originating from three countries showed the non‐inferiority of sole iGM‐CSF treatment. Fibrosis negatively affects response to treatment and survival.