Pulmonary Alveolar Proteinosis in Greece–Türkiye–Cyprus: Answers in a Real-Life Comparison

Papiris, Spyros; Chousein, Efsun; Kallieri, Maria; Mogulkoc, Nesrin; Papaioannou, Andriana; Frangopoulos, Frangiskos; Adamide, Tonia; Kolilekas, Lykourgos; Gökçe, Burak; Ocal, Nesrin; Papakosta, Despina; Özyürek, Berna; Tzilas, Vasilios; Yalnız, Enver; Kontonasiou, Georgia; DEMİRDÖĞEN, EZGİ; Verykakis, Emmanouil; URSAVAŞ, AHMET; Antonogiannaki, Elvira-Markela; Deniz, Pelin; Apollonatou, Vasiliki; Hanta, Ismail; Chatzis, Dimitrios; Levounets, Anastasia; Olympiou, Nicoletta; Vandorou, Kalliopi; Dimeas, Ilias; Daniil, Zoe; Roussakis, Nikolaos; Schams, Andrea; Monnier, Delphine; Rampiadou, Christina; Steiropoulos, Paschalis; Markopoulou, Katerina; Tzanakis, Nikolaos; Gourgoulianis, Konstantinos; Prountzos, Spyridon; Bouros, Demosthenes; Carey, Brenna; Trapnell, Bruce; Griese, Matthias; Çetinkaya, Erdoğan; Manali, Effrosyni

doi:10.1002/resp.70288

Pulmonary Alveolar Proteinosis in Greece–Türkiye–Cyprus: Answers in a Real-Life Comparison

Papiris S. A., Chousein E. G. U., Kallieri M., Mogulkoc N., Papaioannou A. I., Frangopoulos F., ...Daha Fazla

Respirology, 2026 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Basım Tarihi: 2026
Doi Numarası: 10.1002/resp.70288
Dergi Adı: Respirology
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE, Academic Search Ultimate (EBSCO), Biomedical Reference Collection: Corporate Edition (EBSCO), Health Research Premium Collection (ProQuest)
Anahtar Kelimeler: autoantibodies against granulocyte macrophage-colony stimulating factor, autoimmune pulmonary alveolar proteinosis, inhaled granulocyte macrophage-colony stimulating factor, whole lung lavage
Bursa Uludağ Üniversitesi Adresli: Evet

Özet

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), SatO2 (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.