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Abe S.,Tokyo Hiroo Metropolitan General Hospital
Japanese Journal of Chest Diseases | Year: 2014

Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF), or AE-IPF, has been increasingly recognized as a critical event in the clinical course of IPF. AE-IPF is pathologically characterized by diffuse alveolar damage (DAD), which is also observed in acute respiratory distress syndrome (ARDS). Current therapies, including high-dose corticosteroid and immunosuppressive agents, provide little benefit for the poor prognosis in AE-IPF. Recent reports have suggested beneficial effects of anti-coagulant therapy and PMX (polymyxin B-immobilized fiber) treatment in patients with AE-IPF.

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