%0 Journal Article
%T Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.
%A Mabo A
%A Borie R
%A Wemeau-Stervinou L
%A Uzunhan Y
%A Gomez E
%A Prevot G
%A Reynaud-Gaubert M
%A Traclet J
%A Bergot E
%A Cadranel J
%A Marchand-Adam S
%A Bergeron A
%A Blanchard E
%A Bondue B
%A Bonniaud P
%A Bourdin A
%A Burgel PR
%A Hirschi S
%A Marquette CH
%A Quétant S
%A Nunes H
%A Chenivesse C
%A Crestani B
%A Guirriec Y
%A Monnier D
%A Ménard C
%A Tattevin P
%A Cottin V
%A Luque Paz D
%A Jouneau S
%A  
%J Thorax
%V 79
%N 1
%D 2023 12 15
%M 37758458
%F 9.102
%R 10.1136/thorax-2023-220040
%X Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease, predisposing to an increased risk of infection. A complete picture of these infections is lacking.<BR>Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.<BR>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.<BR>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.<BR>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.