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Semin Respir Crit Care Med 2012; 33(05): 433-439
DOI: 10.1055/s-0032-1325154
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Idiopathic Pulmonary Fibrosis

Jessica M. Adkins
1   Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, San Francisco, San Francisco, California
,
Harold R. Collard
1   Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, San Francisco, San Francisco, California
› Author Affiliations
Further Information

Publication History

Publication Date:
21 September 2012 (online)

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Abstract

Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive, chronic fibrosing interstitial lung disease occurring primarily in older adults and limited to the lungs. The prognosis is dire, with half of all patients progressing to death from respiratory failure within 3 to 5 years from initial diagnosis. The pathogenesis of IPF is complex and incompletely understood, and the natural history of this disease is variable and unpredictable. There is no widely accepted treatment for IPF, except for lung transplantation. Nevertheless, these are important and exciting times for the diagnosis and management of patients with IPF. Novel diagnostic approaches (e.g., biomarker-based), improved prognostic models, and clinical trials of novel drug agents may alter disease management substantially over the next few years. For now, clinicians should practice comprehensive management, including symptom-based management, aggressive management of comorbidities, and patient education and support.

Keywords

idiopathic pulmonary fibrosis - usual interstitial pneumonia - familial interstitial pneumonia
 

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