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Pharmacotherapy of Acute Exacerbation of IPF (Corticosteroids, Immunosuppressants, and Direct Hemoperfusion with Polymyxin)

Are High-Dose Steroid Therapy, Other Immunosuppressant Therapy, and PMX Therapy (Often Used in Japan) Really Effective?

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Idiopathic Pulmonary Fibrosis

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Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) carries a rather poor prognosis. The condition has been treated using high-dose steroids, immunosuppressants (cyclosporine A, tacrolimus, cyclophosphamide, azathioprine), anticoagulants (heparin, recombinant human soluble thrombomodulin), neutrophil elastase inhibitors (sivelestat), etc. Recently, direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX-DHP) has been used increasingly in combination with these medications. However, most reports on the efficacy of these treatment methods are based on small-scale retrospective studies, and there is no report of randomized controlled trials (RCTs) demonstrating their efficacy. Thus, there is little scientific evidence of the efficacy of these treatment methods, and no AE-IPF treatment method of high evidence level is included in the guidelines available at present. It would be desirable to facilitate clarification of the pathophysiology of AE-IPF and to carry out RCTs with the cooperation of multiple facilities.

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  1. Department of Respiratory Medicine, Ibaraki Medical Center, Tokyo Medical University, 3-20-1, Chuou, Ami, Inashiki, Ibaraki, 300-0395, Japan

    Masayuki Itoh

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  1. Department of Respiratory Medicine, Tokyo Medical University, Ibaraki Medical Center , Ibaraki, Japan

    Hiroyuki Nakamura

  2. Department of Respiratory Medicine , Tokyo Medical University, Ibaraki Medical Center, Ibaraki, Japan

    Kazutetsu Aoshiba

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Itoh, M. (2016). Pharmacotherapy of Acute Exacerbation of IPF (Corticosteroids, Immunosuppressants, and Direct Hemoperfusion with Polymyxin). In: Nakamura, H., Aoshiba, K. (eds) Idiopathic Pulmonary Fibrosis. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55582-7_12

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