Pulmonary Alveolar Proteinosis in Greece-Türkiye-Cyprus: Answers in a Real-Life Comparison.
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Papiris Spyros A, Chousein Efsun Gonca Uğur, Kallieri Maria, Mogulkoc Nesrin, Papaioannou Andriana I, Frangopoulos Frangiskos, Adamide Tonia, Kolilekas Lykourgos, Gökçe Burak, Ocal Nesrin, Papakosta Despina, Özyürek Berna Akıncı, Tzilas Vasilios, Yalnız Enver, Kontonasiou Georgia, Demirdöğen Ezgi, Verykakis Emmanouil, Ursavaş Ahmet, Antonogiannaki Elvira-Markela, Deniz Pelin Pınar, 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 D
Аннотация
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.