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Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking., Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected from medical records using a standardised questionnaire., Results: 61 patients were included in the final analysis. We identified 5 patients (8%) with fibrosis on initial computed tomography (CT) and 16 patients (26%) with fibrosis on final CT after a median time of 3.6 years. Dust exposure was associated with pulmonary fibrosis occurrence (OR 4.3; p=0.038). aPAP patients treated with whole-lung lavage, rituximab or granulocyte-monocyte colony-stimulating factor therapy did not have more fibrotic evolution than patients who did not receive these treatments (n=25 out of 45, 57% versus n=10 out of 16, 62%; p=0.69). All-cause mortality was significantly higher in fibrotic than in nonfibrotic cases (n=4 out of 16, 25% versus n=2 out of 45, 4.4%; p=0.036, respectively)., Conclusion: In our population, a quarter of aPAP patients progressed towards pulmonary fibrosis. Dust exposure seems to be an important factor associated with this complication. More studies are needed to analyse precisely the impact of dust exposure impact, especially silica, in patients with aPAP., Competing Interests: Conflict of interest: C. Chenivesse declares having received grants from AstraZeneca, GlaxoSmithKline, Novartis and Santelys, personal fees from ALK-Abello, AstraZeneca, Boehringer Ingelheim, Chiesi, GlaxoSmithKline and Sanofi, and congress support from AstraZeneca, Boehringer Ingelheim, Chiesi, Novartis and Sanofi. M. Lederlin has received fees, funding or reimbursement for national and international conferences, boards, expert or opinion groups and research projects over the past 5 years from AstraZeneca, Boehringer, Fresenius-Kabi and Siemens Healthcare. S. Jouneau has received fees, funding or reimbursement for national and international conferences, boards, expert or opinion groups, and research projects over the past 5 years from Actelion, AIRB, AstraZeneca, Bellorophon Therapeutics, Biogen, BMS, Boehringer, Chiesi, Fibrogen, Gilead, GSK, LVL, Mundipharma, Novartis, Olam Pharm, Pfizer, Pliant Therapeutics, Roche and Savara-Serendex. The other authors have nothing to disclose., (Copyright ©The authors 2024.)

Background: Nocardiosis, a bacterial opportunistic infection caused by Nocardia spp, has recently been reported in patients with anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies, but insufficient data are available about disease presentation, outcomes, and occurrence of autoimmune pulmonary alveolar proteinosis (aPAP) in this population., Methods: We performed a prospective, multicenter, nationwide study in France and included patients with a Nocardia infection who had anti-GM-CSF autoantibodies. We describe their clinical, microbiological, and radiological characteristics, and their outcome at 1 year of follow-up., Results: Twenty patients (18 [90%] male) were included, with a median age of 69 (interquartile range, 44-75) years. The organs most frequently involved were the brain (14/20 [70%]) and the lung (12/20 [60%]). Half of the infections were disseminated (10/20 [50%]). Nocardia identification was predominantly made in abscess fluid (17/20 [85%]), among which 10 (59%) were brain abscesses. The 1-year all-cause mortality was 5% (1/20), and only 1 case of aPAP (1/20 [5%]) occurred during the follow-up period., Conclusions: Nocardiosis with anti-GM-CSF autoantibodies is associated with a low mortality rate despite a high incidence of brain involvement. Although the occurrence of aPAP was infrequent during the 1-year follow-up period, long-term clinical data are needed to fully understand the potential relationship between nocardiosis, anti-GM-CSF autoantibodies, and aPAP., (© The Author(s) 2024. Published by Oxford University Press on behalf of Infectious Diseases Society of America.)