Abstract

Aim and objective: to describe treatment modalities in the Greek cohort of aPAP. Methods: retrospective analysis of aPAP patients diagnosed, treated and followed-up in our department from 2002 to 2022. Results: Diagnosed are 41(5 lost to follow-up, 3/5 treated with sc-GMCSF). Analyzed are 36, 22 (61.1%) females, 21 (58.3%) ever-smokers, median (IQR) age at diagnosis 40.0 (32.5-47.0) years, FVC, DLCO (%predicted) and SatO2 80.2 (61.4-96.0), 44.0 (33.0-66.0), 95 (88.0-97.0) [min 52.0-max 98.0] respectively. Follow-up for 24.5 (12-87) [min 2.0-max 240.0] months. Treated with inhaled(i)-GM-CSF were 21(58.3%) by specific protocol including dose de-escalation to lowest-effective after remission and treatment suspension after consolidated disease-free period. All patients treated (both sc or i-GM-CSF) remitted except 2 who developed progressive fibrosis. Whole Lung Lavage (WLL) was performed in 11 (30.5%)(1-65 times) mostly abroad, with diminishing frequency the last 10 years. In all FVC%, DLCO% and SAO2 significantly improved (p=0.013, p=0.013, p=0.003 respectively). Two remitted spontaneously. Infections were in 4/36 (11.1%): MAC, Nocardia asteroides, Pneumocystis jirovecii, Streptococcus Pneumoniae. Four died: 1 respiratory failure, 2 pulmonary fibrosis (PF), 1 lung cancer and PF. During the pandemic, 24 (66.7%) patients received at least 2 vaccination doses against SARS-CoV-2 and 18/32 (56.25%) patients were infected [7 before vaccination]. No COVID-19 related death was reported. Conclusion: In Greece i-GM-CSF, administered according to a specific protocol, is the mainstay of treatment for aPAP patients because of its effectiveness irrespective of disease severity at diagnosis, a real step ahead from WLL.