Learn about prescription IPF treatment
Introduction to IPF
Introduction to IPF
There Are Over 150 Disorders that Comprise the Category of Interstitial Lung Disease
Used with permission from the American Thoracic Society. Available at http://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf.
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The cause may also be unknown (idiopathic interstitial pneumonias)
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1. Raghu G, Brown K. Clin Chest Med. 2004;25:409-419.
Differentiating Between the Various ILDs Is Challenging
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1. Raghu G, Brown K. Clin Chest Med. 2004;25:409-419. 2. Meyer KC. Trans Respir Med. 2014;2:1-13.
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IPF Is the Most Common Idiopathic Interstitial Pneumonia3Interstitial lung disease
Idiopathic interstitial pneumonias
Hypersensitivity pneumonitis
Sarcoidosis
Connective tissue disease-ILD
Unclassifiable idiopathic interstitial pneumonias
Rare idiopathic interstitial pneumonias
Desquamative interstitial pneumonia
Idiopathic pleuroparenchymal fibroelastosis
Idiopathic lymphoid interstitial pneumonia
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Adapted from American Thoracic Society4; Travis WD et al.5ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis.
Other forms of ILD
Idiopathic pulmonary fibrosis
Cryptogenic organizing pneumonia
Idiopathic nonspecific interstitial pneumonia
Major idiopathic interstitial
pneumonias
Respiratory bronchiolitis-interstitial lung disease
Acute interstitial pneumonia
Overview of IPF
and
or
7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 8. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.
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IPF Is a Debilitating Lung Disease5
Fatal lung disease affects adults >60 years of age,8 with a median survival of 3.8 years from diagnosis, generally the shortest of all ILDs9a
IPF is more common in men than women9
Most patients with IPF have a history of cigarette smoking7,8
The majority of patients experience a progressive decline, though the disease course is variable7
As fibrosis progresses, patients will become more breathless, less mobile, and less independent10
7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 8. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 9. Raghu G et al. Lancet Respir Med. 2014;2:566-572. 10. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
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aIn the Medicare population.
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Risk Factors for IPF
7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 8. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 11. Garcia-Sancho C et al. Respir Med. 2011;105:1902-1907.
12. King TE Jr et al. Lancet. 2011;378:1949-1961. 13. Camelo A et al. Front Pharmacol. 2014;4:173.
It is hypothesized that these environmental and genetic risk factors contribute to epithelial cell injury and apoptosis during disease progression12,13
See About IPF: Risk Factors section for more detailed information.
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The Clinical Course of IPF Is Variable and Unpredictable7,14,15
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Worsening of IPF may occur slowly or rapidly, and the disease course may be unexpectedly accelerated by acute exacerbations7,14,15
Acute exacerbations may occur at any time during the course of the disease7
Stable
Time
Disease Progression
Rapid Progression
Slow Progression
Stable
Acute Worsening
Slow, characterized by slowly declining lung function and survival lasting several years14,15
Rapid, characterized by more rapid onset of symptoms and a faster decline in lung function14,15
Episodes of acute worsening may disrupt the course of IPF14
Reprinted with permission of the American Thoracic Society. © 2015 American Thoracic Society. Raghu G et al.7
7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 14. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440. 15. Selman M et al. PLoS One. 2007;2(5):e482.
See About IPF: Disease Course section for more detailed information.
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Common Symptoms Significantly Impact Quality of Life in Patients with IPF10
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Dyspnea
Cough
Fatigue
Depression
10. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61. 16. Olson A et al. Patient Related Outcome Measures. 2016:7;29-35.
17. Lee JS et al. Curr Opin Pulm Med 2011;17(5):348-354.
Dyspnea is universal in patients with IPF17 and is extremely distressing10
Chronic cough causes significant impairment in patients’ lives10
Fatigue/exhaustion occurs frequently,10 and is exacerbated by further muscle deconditioning17
Patients may experience fear, worry, anxiety, and panic related to their poor prognosis10,17
Dyspnea and physical function capacity are strong drivers of health-related quality of life16
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Difficulty with Diagnosis Is Common in Patients With IPF14,18,19
Difficulty with diagnosis is common, as the early symptoms of IPF are nonspecific and the disease itself is rare20,21
of patients report seeing 3 or more physicians before receiving a diagnosisof IPF21
38%
of patients are initially misdiagnosed21
>50%
delay between onset of symptoms and diagnosis14,18,19
1-2 year
14. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440. 18. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
19. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644. 20. Cordier JF et al. Eur Respir J. 2013;42(4):916-923. 21. Collard HR et al. RespirMed. 2007;101:1350-1354.
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COPD, chronic obstructive pulmonary disease.
The Diagnostic Process Can Take an Average of 1-2 Years14, 18-19
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Bronchitis, asthma, COPD, emphysema, or heart disease are often considered first, as symptoms of IPF are common manifestations of these diseases21
1-2 Years (average)14, 18-19
Symptom Onset
Diagnosis
Adapted from Lamas DJ et al.22
aDelay is defined as time from onset of dyspnea to time of initial evaluation at a tertiary care center.
14. Ley B et al. Am J RespirCrit Care Med. 2011;183(4):431-440. 18. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
19. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644. 21. Collard HR et al. Respir Med. 2007;101(6):1350-1354. 22. Lamas DJ et al. Am J Respir Crit Care Med. 2011;184:842-847.
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There Are Several Diagnostic Tools for Evaluating Interstitial Lung Disease
Clinical Assessment
SurgicalAssessment
Radiological Assessment
7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 8. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 23. Wanger J et al. Eur Respir J. 2005;26:511-522. 24. Pulmonary Fibrosis Foundation Patient Information Guide. Available at http://www.pulmonaryfibrosis.org/docs/default-source/patient-information-guides/2017-pff-patient-information-guide.pdf?sfvrsn=0. Accessed July 12, 2017.
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Multidisciplinary Meeting
See Diagnosing IPF: Diagnostic Criteria and Diagnostic Algorithm section for more detailed information.
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FEV1, forced expiratory volume in 1 second.
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Recommendations include:
- The majority of patients should begin pulmonary rehabilitation7
- Patients with clinically significant resting hypoxemia should begin long-term oxygen therapy7
- Patients should be considered and evaluated for lung transplant in a timely manner7
- All patients should be made aware of available medications and clinical trials for possible enrollment7
- Patients should receive a yearly flu vaccine25
Recommendations for Management of IPF
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See Managing Patients: IPF Symptom Management section for more detailed information.
Comprehensive Care for Patients with IPF Includes Several Patient-Centric Areas
26. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
Disease-centered management
Symptom-centered management
Support-centered management
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See Managing Patients: Support section for more detailed information.
Summary
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8. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.14. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440. 17. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354. 18. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032. 19. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644. 26. Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816. 27. Kubo H et al. Chest. 2005;128:1475-1482. 28. Kim DS et al. Eur Respir J. 2006;27:143-150. 29. Daniels CE et al. Eur Respir J. 2008;32:170-174. 30. Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910.
References
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