Pulmonary fibrosis affects 1 in 4 patients with autoimmune PAP, study finds
Pulmonary fibrosis develops in about a quarter of patients with autoimmune pulmonary alveolar proteinosis (aPAP) over time, and dust exposure is a significant risk factor for this progression, according to a study.
Data from 61 patients newly diagnosed with aPAP were analyzed in the retrospective cohort study. Initial imaging found fibrosis in 8% of patients, increasing to 26% after a median follow-up of 3.6 years. Dust exposure was a significant risk factor for fibrosis development, with an odds ratio of 4.3 (P = 0.038).
Treatment with whole-lung lavage, rituximab, or granulocyte-monocyte colony-stimulating factor did not appear to influence fibrotic progression. However, fibrosis was linked to worse outcomes, with higher all-cause mortality observed in fibrotic cases compared to non-fibrotic cases (25% vs 4.4%, P = 0.036).
Reference
Guirriec Y, Luque-Paz D, Bernard G, et al. Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study. ERJ Open Res. 2024;10(6):00314-2024. doi: 10.1183/23120541.00314-2024. PMID: 39624377; PMCID: PMC11610044.
