aDivision of Pulmonary and Critical Care Medicine, Weill Cornell Medical College, New York, NYbDivision of Pulmonary and Critical Care Medicine, and Idiopathic Pulmonary Fibrosis Foundation Clinical Care Site, University of Michigan, Ann Arbor, MI
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As seen in this CME online activity (available at http://courses.elseviercme.com/chest16/647e), idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrotic lung disease of unknown cause. It is the most common form of idiopathic interstitial pneumonia in adults. Its prevalence among US Medicare beneficiaries has more than doubled in the past 14 years. With the approval of two agents that reduce functional decline and disease progression, and the 2015 update of the American Thoracic Society guidelines on the treatment of IPF, the options for appropriate clinical management of the disease have become well defined. Early diagnosis and early initiation of treatment are of critical importance for long-term clinical outcomes. The diagnostic process, which may require a multidisciplinary team of experts, is centered on excluding systemic diseases and exposures and identifying a pattern of usual interstitial pneumonia on high-resolution CT or surgical lung biopsy results. Familiarity with the 2015 American Thoracic Society guidelines is critical with respect to both the treatments that the guidelines recommend and those that they advise against. Standard immunosuppressive therapy is no longer indicated, whereas pirfenidone, nintedanib, and antacid therapy are all conditionally recommended for use. Individualizing treatment is important in light of potential improved adherence to both drug therapy and health behaviors. An early referral to an interstitial lung disease center offers the advantages of comprehensive diagnostic and disease-management expertise, potential enrollment in a clinical trial, and evaluation for transplantation.