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Pharmacologic Treatments for Idiopathic Pulmonary Fibrosis

Clinician Reviews. 2018 April;28(4):16-21
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This chronic disease has historically lacked an effective treatment option, but the FDA recently approved two: pirfenidone and nintedanib. This discussion of these new drug therapies—including mechanism of action, study results, and adverse effects—could help your patients catch their breath.

TREATMENT HISTORY

IPF has a long history of tried and failed treatment options. The American Thoracic Society (ATS), in concert with other professional organizations, has published comprehensive guidelines and recommendations pertaining to the use of pharmacologic medications to control disease progression. Warfarin and other anticoagulants have been studied, based on the observation that a procoagulant state promotes fibrotic changes in the lung tissue.13 However, anticoagulant use is not recommended in patients with IPF due to lack of efficacy and high potential for harm.13

Immunosuppressants have also been in the spotlight as possible treatment for IPF, but a clinical study investigating the efficacy of a three-drug regimen including prednisone, azathioprine, and N-acetylcysteine was stopped early due to increased risk for harm. Endothelin antagonists and potent tyrosine kinase inhibitors are also not recommended in the most recent edition of IPF guidelines, as they lack benefit.13

In fact, prior to the 2015 edition of the guidelines, no single medication was routinely recommended for patients with IPF. But this is now changing, following the 2014 FDA approval of two new drugs, nintedanib and pirfenidone, designed specifically to treat IPF.14 These drugs have shown promise in clinical trials (results of which are summarized in Table 2).

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