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Idiopathic Pulmonary Fibrosis Clinical Presentation

  • Author: Amanda M K Godfrey, MD; Chief Editor: Ryland P Byrd, Jr, MD  more...
 
Updated: Aug 11, 2015
 

History

The clinical symptoms of idiopathic pulmonary fibrosis 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 (IPF), 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.[14] 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.[14]

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.[26] 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).[27] 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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Physical

In most patients with idiopathic pulmonary fibrosis, the physical examination reveals fine bibasilar inspiratory crackles (Velcro crackles). Additionally, digital clubbing is seen in 25-50% of patients with idiopathic pulmonary fibrosis (IPF).[14] Extrapulmonary involvement does not occur with idiopathic pulmonary fibrosis, and, therefore, physical examination findings do not help to confirm the diagnosis.

Pulmonary hypertension is a common comorbidity in patients with idiopathic pulmonary fibrosis, and an estimated 20-40% of patients with idiopathic pulmonary fibrosis who are evaluated or listed for lung transplantation have pulmonary hypertension at rest.[6] Physical examination findings may be suggestive of the presence of pulmonary hypertension. Patients may have a loud P2 component of the second heart sound, a fixed split S2, a holosystolic tricuspid regurgitation murmur, and pedal edema. As right ventricular hypertrophy ensues, a right ventricular heave may be palpated at the lower left sternal border and increased right atrial pressure may cause elevation of the jugular venous pressure.[7]

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Causes

The etiology of idiopathic pulmonary fibrosis remains undefined; however, in the current hypothesis regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), exposure to an inciting agent (eg, smoke, environmental pollutants, environmental dust, viral infections, gastroesophageal reflux disease, chronic aspiration) in a susceptible host may lead to the initial alveolar epithelial damage.[17] This damage may lead to activation of the alveolar epithelial cells, which provokes the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblastic foci, leading to the exaggerated accumulation of extracellular matrix with the irreversible destruction of the lung parenchyma.[17]

Other potential causes of idiopathic pulmonary fibrosis have been recognized through the study of familial pulmonary fibrosis. Familial pulmonary fibrosis, affecting 2 or more members of the same primary biological family, accounts for less than 5% of total patients with idiopathic pulmonary fibrosis.[28]

Genetic mutations in serum surfactant protein C have been discovered in some individuals with familial pulmonary fibrosis.[28] These mutations in serum surfactant protein C may damage type II alveolar epithelial cells.[28] Additionally, a common variant in the putative promoter of the gene encoding mucin 5B (MUC5B) has been associated with the development of both familial interstitial pneumonia and sporadic pulmonary fibrosis.[21]

Finally, mutant telomerase is associated with familial idiopathic pulmonary fibrosis.[20] Pulmonary fibrosis in patients with short telomeres is provoked by a loss of alveolar epithelial cells. Telomere shortening also occurs with aging and can also be acquired. This telomere shortening could promote the loss of alveolar epithelial cells, resulting in aberrant epithelial cell repair, and therefore should be considered as another potential contributor to the pathogenesis of idiopathic pulmonary fibrosis.[20]

Respiratory viruses have been considered a particularly likely cause of AE-IPF based on the similarities in clinical and radiologic presentation between and AE-IPF and viral pneumonitis and the poor sensitivity of standard methods of viral detection. A study by Wootton et al used genomics-based discovery methods to define the role of viral infections in AE-IPF. Initial multiplex polymerase chain reaction (PCR) revealed common respiratory viral infection in only 4 of 43 patients with AE-IPF. Pan-viral microarrays revealed torque teno virus (TTV) in 12 patients with AE-IPF. The pathogenic significance of TTV in AE-IPF is unclear. Overall, viral infection was not detected in most cases of AE-IPF.[29]

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Contributor Information and Disclosures
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; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

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

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.

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

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

Disclosure: Nothing to disclose.

Chief Editor

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

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

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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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.
Table 1. Scoring for mortality risk in IPF.
  Predictor Points
Sex Female 0
Male 1
Age (years) ≥60 0
61-65 1
>65 2
FVC (% predicted) >75 0
50-75 1
< 50 2
DLCO (% predicted) >55 0
36-55 1
≤35 2
Cannot perform 3
Table 2. Staging and mortality risk for IPF.
Stage I II III
Points 0-3 4-5 6-8
Mortality      
1-year 5.6 16.2 39.2
2-year 10.9 29.9 62.1
3-year 16.3 42.1 76.8
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