(1) The role of the immune response in the pathogenesis of idiopathic pulmonary fibrosis (IPF) remains controversial. We hypothesized that peripheral blood immune phenotypes will be different in IPF patients and may relate to the disease severity and progression. (2) Whole blood flow cytometry staining was performed at diagnosis in 32 IPF patients, and in 32 age- and smoking-matched healthy controls. Thirty-one IPF patients were followed up for one year and categorized as stable or progressors based on lung function, deterioration and/or death. At 18–60 months, immunophenotypes were characterized again. (3) The main results showed that: (1) compared to matched controls, at diagnosis, patients with IPF showed more neutrophils, CD8+HLA-DR+ and CD8+CD28− T cells, and fewer B lymphocytes and naïve T cells; (2) in IPF, circulating neutrophils, eosinophils and naïve T cells were associated with lung function abnormalities; (3) patients whose disease progressed during the 12 months of follow-up showed evidence of cytotoxic dysregulation, with increased CD8+CD28− T cells, decreased naïve T cells and an inverted CD4/CD8 ratio at baseline; and (4) blood cell alterations were stable over time in survivors. (4) IPF is associated with abnormalities in circulating immune cells, particularly in the cytotoxic cell domain. Patients with progressive IPF, despite antifibrotic therapy, present an over-activated and exhausted immunophenotype at diagnosis, which is maintained over time.
High-risk pulmonary embolism (PE) is a life-threatening condition characterized by he-modynamic instability, often leading to catastrophic outcomes such as cardiac arrest and cardiogenic shock. We conducted a retrospective analysis of patients diagnosed with high-risk PE at a single tertiary center between 2018 and 2024. Catastrophic PE was de-fined as high-risk PE with hemodynamic collapse, including cardiac arrest and/or the requirement for high-dose vasopressors. Data on clinical characteristics, treatments, and outcomes were analyzed. Catastrophic PE accounted for 59% of cases. Systemic throm-bolysis was the most frequent reperfusion strategy (67%), while catheter-directed therapies (35.4%) and VA-ECMO (11.4%) were used selectively. Despite aggressive management, catastrophic PE exhibited significantly higher mortality rates at 7 days (40%) and 30 days (49%) compared to non-catastrophic cases (9% and 12.5%, respectively). These patients also showed higher rates of multiorgan failure and required more invasive support. This study underscores the importance of early recognition and tailored treatment strategies for catastrophic PE, highlighting its distinct clinical presentation and worse outcomes compared to non-catastrophic high-risk PE. Further research is essential to refine treatment protocols and improve survival in this critically ill population, emphasizing the utility of a standardized classification to enhance clinical management and research consistency.
High-risk pulmonary embolism (PE) is a life-threatening condition characterized by hemodynamic instability, often leading to catastrophic outcomes such as cardiac arrest and cardiogenic shock. We conducted a retrospective analysis of patients diagnosed with high-risk PE at a single tertiary center between 2018 and 2024. Catastrophic PE was defined as high-risk PE with hemodynamic collapse, including cardiac arrest and/or the requirement for high-dose vasopressors. Data on clinical characteristics, treatments, and outcomes were analyzed. Catastrophic PE accounted for 59% of cases. Systemic thrombolysis was the most frequent reperfusion strategy (67%), while catheter-directed therapies (35.4%) and VA-ECMO (11.4%) were used selectively. Despite aggressive management, catastrophic PE exhibited significantly higher mortality rates at 7 days (40%) and 30 days (49%) compared to non-catastrophic cases (9% and 12.5%, respectively). These patients also showed higher rates of multiorgan failure and required more invasive support. This study underscores the importance of early recognition and tailored treatment strategies for catastrophic PE, highlighting its distinct clinical presentation and worse outcomes compared to non-catastrophic high-risk PE. Further research is essential to refine treatment protocols and improve survival in this critically ill population, emphasizing the utility of a standardized classification to enhance clinical management and research consistency.