Abstract
BACKGROUND/AIMS: Patients with acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) typically have a poor prognosis; however, no effective treatment is available. In recent years, several retrospective studies have suggested the clinical benefits of direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP) in patients with AE-IPF. Herein, we aimed to investigate the efficacy and safety of PMX-DHP treatment in patients with AE-IPF. METHODS: Patients diagnosed with AE-IPF (n = 10) with a partial pressure of oxygen to fraction of inspiratory oxygen ratio (P/F ratio) > 100 were prospectively enrolled at a single center. PMX-DHP was performed twice for 6 hours (at 24-h intervals) at a flow rate of 80-100 mL/min, and steroid pulse therapy was concurrently administered (500 mg of methylprednisolone for 3 d). RESULTS: The mean patient age was 67 years, and 80.0% were male. During the follow-up (median, 42.5 d; interquartile range, 16.0-174.0 d), seven (70.0%) patients died (including two who underwent transplantation); the in-hospital mortality rate was 70%, while the 30- and 90-day mortality rates were 50.0% and 70.0%, respectively. After 48 hours of PMX-DHP treatment, the P/F ratio improved (mean, 160.0 vs. 229.0; p = 0.054) and C-reactive protein level decreased (mean, 8.3 mg/dL vs. 3.5 mg/dL; p = 0.012). During hospitalization, no PMX-DHP-associated adverse events were observed. CONCLUSION: Our results suggest that PMX-DHP treatment may be useful at improving oxygenation and reducing inflammation in patients with AE-IPF with acceptable safety profiles, however without affecting their prognosis.