Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that can progress to pulmonary fibrosis, but data on fibrosis prevalence and associated risk factors are limited.
This retrospective, multicentre, nationwide cohort study included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected from medical records using a standardised questionnaire.
A total of 61 patients were included in the final analysis. Pulmonary fibrosis was identified in 5 patients (8%) on initial computed tomography (CT) and in 16 patients (26%) on final CT after a median follow-up of 3.6 years. Dust exposure was significantly associated with the occurrence of pulmonary fibrosis (odds ratio 4.3; p=0.038).
In this cohort, one-quarter of aPAP patients developed pulmonary fibrosis, with dust exposure emerging as a significant risk factor. Further studies are needed to investigate the impact of dust exposure, particularly silica, in aPAP patients. Read the full article here.