What are the history findings of idiopathic pulmonary fibrosis (IPF)?

Answer

The clinical symptoms of idiopathic pulmonary fibrosis (IPF) are nonspecific. Most patients present with exertional dyspnea and a nonproductive cough. Such symptoms can be shared with a variety of pulmonary and cardiac diseases. Dyspnea, which is the most prominent symptom in idiopathic pulmonary fibrosis, usually begins insidiously and is often progressive. Associated systemic symptoms can occur but are not common. Some of these systemic symptoms include weight loss, low-grade fevers, fatigue, arthralgias, or myalgias.

The reported median duration of symptoms before the diagnosis of idiopathic pulmonary fibrosis is established is one to two years.^[12]Most patients are referred to a cardiologist for evaluation of exertional dyspnea prior to being referred to a pulmonologist. Approximately 5% of patients have no presenting symptoms when idiopathic pulmonary fibrosis is diagnosed. Among asymptomatic patients with idiopathic pulmonary fibrosis (diagnosed by radiographic abnormalities found on routine chest radiograph screening and lung biopsy showing usual interstitial pneumonia), symptoms developed approximately 1000 days after the recognition of the radiographic abnormality.^[12]

It is critical to obtain a complete history, including medication history, drug use, social history, occupational, recreational, and environmental respiratory exposure history, risk factors for human immunodeficiency virus infection, and review of systems, to ensure other causes of interstitial lung disease are excluded. Amiodarone, bleomycin, and nitrofurantoin are notable medications associated with pulmonary fibrosis. Oxidant stress from smoking may damage alveolar epithelial cells and contribute to the pathogenesis of idiopathic pulmonary fibrosis.^[36]Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit. Any prior exposure to asbestos, silica, heavy metals, contaminated ventilation systems, moldy foliage, and/or pigeon droppings should be investigated. Evidence of arthralgia, arthritis, photosensitivity, Raynaud phenomenon, dry eyes, and/or dry mouth on review of systems may indicate the presence of a collagen-vascular disease.

Physicians should pay attention to historical clues that may suggest the presence of obstructive sleep apnea (OSA) because a 2009 study demonstrated the high prevalence of OSA in patients with idiopathic pulmonary fibrosis. Fifty outpatients with stable idiopathic pulmonary fibrosis were prospectively evaluated for the presence of OSA. OSA was defined as an apnea-hypopnea index (AHI) of greater than 5 events per hour. Ten subjects (20%) had mild OSA (AHI of 5-15 events per hour) and 34 subjects (68%) had moderate-to-severe OSA (AHI of >15 events per hour).^[37]Therefore, the prevalence of OSA in this sample was 88%, suggesting that OSA in patients with idiopathic pulmonary fibrosis may have been previously underrecognized.

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Media Gallery

Chest radiograph of a patient with idiopathic pulmonary fibrosis showing bilateral lower lobe reticular opacities (red circles).
Classic subpleural honeycombing (red circle) in a patient with a diagnosis of idiopathic pulmonary fibrosis.
A patient with IPF and a confirmed histologic diagnosis of usual interstitial pneumonia. Note the reticular opacities (red circle) distributed in both lung bases and the minimal ground-glass opacities (blue circle).
A patient with nonspecific interstitial pneumonia. Note the predominance of ground-glass opacities (blue circles) and a few reticular lines (red arrow).
Patchwork distribution of abnormalities in a classic example of usual interstitial pneumonia (low-magnification photomicrograph; hematoxylin and eosin stain; original magnification, X4). Courtesy of Chad Stone, MD.

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Author

Amanda M K Godfrey, MD Associate Staff, Department of Internal Medicine, IHA Pulmonary, Critical Care, and Sleep Consultants; Associate Staff, Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, St Joseph Mercy Ann Arbor

Amanda M K Godfrey, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Michigan State Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Medical Director, Pulmonary Medicine General Practice Unit (F2), Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Guy W Soo Hoo, MD, MPH Clinical Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Medical Intensive Care Unit, Pulmonary and Critical Care Section, West Los Angeles Healthcare Center, Veteran Affairs Greater Los Angeles Healthcare System

Guy W Soo Hoo, MD, MPH is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society, Society of Critical Care Medicine, California Thoracic Society, American Association for Respiratory Care

Disclosure: Nothing to disclose.

Additional Contributors

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP Thomas H Davis Chair in Pulmonary Medicine, Chief, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Professor of Internal Medicine, Pediatrics, and Translational Science, Associate Director, Center for Genomics and Personalized Medicine Research, Wake Forest University School of Medicine; Executive Director of the Respiratory Service Line, Wake Forest Baptist Medical Center

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Integrity CE, Merck<br/>Received income in an amount equal to or greater than $250 from: – Array Biopharma, AstraZeneca, Aerocrine, Airsonett AB, Boehringer-Ingelheim, Experts in Asthma, Gilead, GlaxoSmithKline, Merck, Novartis, Ono Pharmaceuticals, Pfizer, PPD Development, Quintiles, Sunovion, Saatchi & Saatichi, Targacept, TEVA, Theron.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Rajesh G. Patel, MD, and Javier I. Diaz, MD, to the development and writing of this article.

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