Background and objective: 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.
Methods: The clinical records of patients with AE admitted to our intensive care unit between 2006 and 2015 were retrospectively reviewed.
Results: 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).
Conclusion: The early induction of PMX-DHP treatment may improve the prognosis of patients with AE, especially those with dermatomyositis.
Keywords: acute exacerbation; dermatomyositis; interstitial pneumonia; polymyxin B-immobilized fibre column-direct hemoperfusion.
© 2017 Asian Pacific Society of Respirology.