Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is recognized as an important cause of mortality. AE has also been reported in patients with other interstitial lung diseases such as idiopathic non-specific interstitial pneumonia (NSIP) and interstitial pneumonia associated with collagen vascular disease (CVD). Current therapies such as high-dose corticosteroid with immunosuppressive agents have provided little benefit for AE. Direct hemoperfusion (DHP) with a polymyxin B-immobilized fibre column (PMX) was originally developed for the treatment of endotoxaemia. Recent clinical reports have suggested beneficial effects of PMX-DHP treatment on patients with AE. In this study, we evaluated the effectiveness and safety of PMX-DHP treatment for patients with AE. The clinical records of patients with AE admitted to our intensive care unit between 2006 and 2015 were retrospectively reviewed. Of 54 patients with AE identified from clinical records, 24 were treated with PMX-DHP and 30 were treated without PMX-DHP. The peripheral white blood cell count was significantly decreased (P < 0.001) and the PaO2 /FiO2 (P/F) ratio was significantly improved after PMX-DHP (P = 0.032). While no significant difference was found in the survival proportion between patients treated with and without PMX-DHP, the prognosis of patients with dermatomyositis was significantly improved with the treatment (P = 0.045). Among the PMX-DHP-treated patients, those who received the treatment within 3 days of AE onset tended to have a better prognosis (P = 0.026). The early induction of PMX-DHP treatment may improve the prognosis of patients with AE, especially those with dermatomyositis.

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