{Reference Type}: Journal Article
{Title}: Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.
{Author}: Mabo A;Borie R;Wemeau-Stervinou L;Uzunhan Y;Gomez E;Prevot G;Reynaud-Gaubert M;Traclet J;Bergot E;Cadranel J;Marchand-Adam S;Bergeron A;Blanchard E;Bondue B;Bonniaud P;Bourdin A;Burgel PR;Hirschi S;Marquette CH;Quétant S;Nunes H;Chenivesse C;Crestani B;Guirriec Y;Monnier D;Ménard C;Tattevin P;Cottin V;Luque Paz D;Jouneau S; ;
{Journal}: Thorax
{Volume}: 79
{Issue}: 1
{Year}: 2023 12 15
{Factor}: 9.102
{DOI}: 10.1136/thorax-2023-220040
{Abstract}: 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.