Direct hemoperfusion with polymyxin B-immobilized fiber column in a patient with acute exacerbation of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by dyspnea and a worsening of the lung function. Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) are defined by a clinically significant respiratory deterioration, that typically develops in less than 1 month, accompanied by new radiologic abnormalities on high-resolution computed tomography, including diffused and bilateral ground-glass opacification, along with an absence of other obvious clinical etiologies. Recently, AE-IPF has gained significant importance as a major cause of mortality and morbidity. However, despite the extremely poor prognosis of the condition, no well-validated therapeutic interventions are currently available. Therefore, novel treatment modalities are being investigated and applied in addition to conventional treatments. Among them, several studies have reported that a direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP), developed for endotoxin removal in septic shock, has an effect on AE-IPF. We describe two cases of PMX-DHP treatment with conflicting results. One patient successfully recovered via a PMX-DHP in severe AE-IPF that required extracorporeal membrane oxygenation (ECMO). PMX-DHP subsequently improved oxygenation (PaO(2)/FiO(2) ratio) and decreased the levels of inflammatory markers (interleukin-6, C-reactive protein, and white blood cells). The patient dramatically recovered without the need for ECMO. PMX-DHP may be considered an alternative therapy in AE-IPF patients requiring mechanical ventilation or ECMO.