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Table 3 Pharmacological therapies for AE-IPF, currently proposed or under investigation

From: Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome?

Therapy

Study

Nintedanib (preventive therapy)

Richeldi et al., 2011 [74]; Richeldi et al., 2014 [75]

Pirfenidone (preventative therapy)

Azuma et al., 2005 [76]; Taniguchi et al., 2010 [77]

Anti-acid therapy (preventative therapy)

Lee et al., 2013 [78]

Corticosteroid monotherapy

Akira et al., 1997 [79]; Al-Hameed and Sharma, 2004 [80]; Suzuki et al., 2011 [81]; Tachikawa et al., 2012 [82]

Cyclophosphamide

Akira et al., 2008 [83]; Fujimoto et al., 2012 [84]; Tachikawa et al., 2012 [82]; Yokoyama et al., 2010 [85]

Cyclosporine

Homma et al., 2005 [86]; Inase et al., 2003 [87]; Sakamoto, et al., 2010 [88]; Fujimoto et al., 2012 [84]; Yokoyama et al., 2010 [85]

Polymyxin-B immobilized fiber column hemoperfusion

Abe et al., 2011 [89]; Abe et al., 2012 [90]; Oishi et al., 2013 [91]; Seo et al., 2006 [92]; Tachibana et al., 2011 [93]

Rituximab, plasma exchange, and intravenous immunoglobulin

Donahoe et al., 2015 [94]

Tacrolimus

Horita et al., 2011 [95]

Thrombomodulin

Kataoka et al., 2015 [96]; Tsushima et al., 2014 [97]; Isshiki et al., 2015 [98]

Cessation of immunosuppression, best supportive care, broad-spectrum antimicrobials: “nonsteroid approach”

Papiris et al., 2015 [73]

  1. AE-IPF acute exacerbation of idiopathic pulmonary fibrosis