Hermansky-Pudlak Syndrome type 1 (HPS-1) is a rare genetic disease especially common in Puerto Rico, and all affected individuals eventually develop pulmonary fibrosis, a serious scarring of the lungs. In this study, researchers examined immune activity in HPS-1 patients of different ages and observed that those over 40 showed signs of increased neutrophil activity—white blood cells involved in inflammation. These patients had higher levels of markers linked to inflammation and tissue injury, including neutrophil extracellular traps (NETs), IL-6, IL-8, and fibrosis-related proteins like MMP-7 and MMP-8. Levels of anandamide, a molecule associated with lung scarring, were also higher in this group and correlated with neutrophil activation markers. These findings suggest that age-related immune changes may play a role in lung damage in HPS-1, highlighting the need for further studies to elucidate this process and explore novel treatments.