PT  - JOURNAL ARTICLE
AU  - Mabo, Axelle
AU  - Borie, Raphael
AU  - Wemeau-Stervinou, Lidwine
AU  - Uzunhan, Yurdagül
AU  - Gomez, Emmanuel
AU  - Prevot, Gregoire
AU  - Reynaud-Gaubert, Martine
AU  - Traclet, Julie
AU  - Bergot, Emmanuel
AU  - Cadranel, Jacques
AU  - Marchand-Adam, Sylvain
AU  - Bergeron, Anne
AU  - Blanchard, Elodie
AU  - Bondue, Benjamin
AU  - Bonniaud, Philippe
AU  - Bourdin, Arnaud
AU  - Burgel, Pierre Regis
AU  - Hirschi, Sandrine
AU  - Marquette, Charles Hugo
AU  - Quétant, Sébastien
AU  - Nunes, Hilario
AU  - Chenivesse, Cécile
AU  - Crestani, Bruno
AU  - Guirriec, Yoann
AU  - Monnier, Delphine
AU  - Ménard, Cédric
AU  - Tattevin, Pierre
AU  - Cottin, Vincent
AU  - Luque Paz, David
AU  - Jouneau, Stéphane
ED - 
TI  - Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort
AID  - 10.1136/thorax-2023-220040
DP  - 2024 Jan 01
TA  - Thorax
PG  - 68--74
VI  - 79
IP  - 1
4099  - http://thorax.bmj.com/content/79/1/68.short
4100  - http://thorax.bmj.com/content/79/1/68.full
SO  - Thorax2024 Jan 01; 79
AB  - Background Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease, predisposing to an increased risk of infection. A complete picture of these infections is lacking.Research question Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.Methods We conducted a retrospective cohort including all patients diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected using a standardised questionnaire including demographics, comorbidities, imaging features, outcomes and microbiological data.Results We included 104 patients, 2/3 were men and median age at diagnosis was 45 years. With a median follow-up of 3.4 years (IQR 1.7–6.6 years), 60 patients (58%), developed at least one infection, including 23 (22%) with opportunistic infections. Nocardia spp was the main pathogen identified (n=10). Thirty-five (34%) patients were hospitalised due to infection. In univariate analysis, male gender was associated with opportunistic infections (p=0.04, OR=3.88; 95% CI (1.02 to 22.06)). Anti-granulocyte macrophage colony-stimulating factor antibody titre at diagnosis was significantly higher among patients who developed nocardiosis (1058 (316–1591) vs 580 (200–1190), p=0.01). Nine patients had died (9%), but only one death was related to infection.Interpretation Patients with aPAP often presented with opportunistic infections, especially nocardiosis, which highlights the importance of systematic search for slow-growing bacteria in bronchoalveolar lavage or whole lung lavage.Data can be made available upon reasonable request.