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.

  • Interstitial lung disease (ILD) is a broad term for a collection of over 150 disorders that inflame or scar the lungs, leading to pulmonary fibrosis
  • May be an acute or chronic process
  • Associated with infection, exposure to dust or other particles, or an underlying genetic predisposition
  •  

     

    2

    The cause may also be unknown (idiopathic interstitial pneumonias)

    2

    1. Raghu G, Brown K. Clin Chest Med. 2004;25:409-419. 

    Differentiating Between the Various ILDs Is Challenging

  • ILDs have similar symptoms, physiology, and features on thoracic imaging1
  • Diagnosis is improved by multidisciplinary discussion among pulmonologists, radiologists, and pathologists2
  • Successful management of a patient with ILD depends on an accurate diagnosis, allowing physicians to1:
  • –Provide patients with appropriate prognostic information
    –Develop a management strategy with the patient

     

    3

    1. Raghu G, Brown K. Clin Chest Med. 2004;25:409-419. 2. Meyer KC. Trans Respir Med. 2014;2:1-13.

    3

    CHANGE_ME

    IPF Is the Most Common Idiopathic Interstitial Pneumonia3
    Interstitial 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
    4
    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

  • IPF is a progressive, fatal lung disease of unknown etiology7,8
  • Fibrosis is limited to the lung alveoli and interstitium7,8
  • A diagnosis of IPF requires8:
  • –Exclusion of other known causes of ILD

    and

    –The presence of a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT)

    or

    –Specific combinations of HRCT patterns and surgical lung biopsy patterns

    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.

     

    5

    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.

    6

    aIn the Medicare population.

    6

    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

  • Several environmental and genetic risk factors have been described despite its unknown etiology7,8
  • Genetic factors have been linked to both sporadic and familial IPF7,8
  • –It is estimated that 5% of cases of IPF are familial7
    –A family history of IPF is a strong risk factor, increasing the odds up to 6-fold11
  • Familial and sporadic IPF are clinically, radiologically, and histologically identical7
  •  

     

     

     

     

    See About IPF: Risk Factors section for more detailed information.

    7

    The Clinical Course of IPF Is Variable and Unpredictable7,14,15

    8

    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.

    8

    Common Symptoms Significantly Impact Quality of Life in Patients with IPF10

    9

    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

    9

    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

    –Symptoms may be attributed to more common ailments such as bronchitis, asthma, COPD, emphysema, or heart disease21

     

     

     

    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.

    10

     

    10

    COPD, chronic obstructive pulmonary disease.

    The Diagnostic Process Can Take an Average of 1-2 Years14, 18-19

    11

    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.

    11

    There Are Several Diagnostic Tools for Evaluating Interstitial Lung Disease

    Clinical Assessment

  • Comprehensive medical history7,8,23
  • Physical examination7
  • –Chest auscultation7
  • Pulmonary function testing7,23:
  • –FVC, FEV1, DLCO, lung volumes
  • 6-minute walk test (6MWT)7
  • Pulse oximetry24
  • Cardiac evaluation to rule out heart disease24
  • Serologic testing7,8
  •  

    SurgicalAssessment

  • Bronchoscopy
  • Surgical lung biopsy (if necessary7,8)
  • Radiological Assessment

  • Chest x-ray24
  • HRCT7,8
  •  

     

    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.

    12

    Multidisciplinary Meeting

  • Team-based approach to evaluating all evidence in order to reach a consensus diagnosis8
  • See Diagnosing IPF: Diagnostic Criteria and Diagnostic Algorithm section for more detailed information.

    12

    FEV1, forced expiratory volume in 1 second.

    CHANGE_ME

     
    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
    13
    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

  • Smoking cessation
  • Comorbidity management
  • Pulmonary rehabilitation
  • Supplemental oxygen
  • FDA-approved pharmacologic therapies
  • Lung transplant
  • Symptom-centered management

  • Chronic cough
  • Dyspnea
  • Fatigue/deconditioning
  • Depression and anxiety
  • Sleep disturbance
  • Support-centered management

  • Educational support
  • Support group services
  • 14

    See Managing Patients: Support section for more detailed information.

    Summary

  • IPF is a progressive, fatal disease that typically affects adults over 60 years of age8,26
  • Patients may experience periods of sudden respiratory decline termed acute exacerbations of IPF,26 which are associated with a high mortality rate27-29
  • Symptoms are nonspecific, but patients often present with chronic exertional dyspnea and a dry, nonproductive cough26
  • –The average time from symptom onset to diagnosis is 1-2 years14,18-19
  • Diagnosis of IPF is difficult, often requiring discussion between pulmonologists, radiologists, and pathologists26,30
  • –Diagnosis requires a comprehensive patient evaluation including a detailed medical history, exclusion of other possible causes of lung disease, pulmonary function testing, HRCT, and in some cases, surgical lung biopsy26
  • Comprehensive care includes disease-focused, symptom-focused, and support-focused management17
  •  

     

    15

    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

    1.Raghu G, Brown K. Clin Chest Med. 2004;25:409-419.
    2.Meyer KC. Trans Respir Med. 2014;2:1-13.
    3.Spagnolo P et al. Multidiscip Respir Med. 2012;7:42.
    4.American Thoracic Society. Am J Respir Crit Care Med. 2002;165:277-304.
    5.Travis WD et al. Am J Respir Crit Care Med. 2013;188(6):733-748.
    6.Ryerson CJ and Collard HR. Curr Opin Pulm Med. 2013;19:453-459.
    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.
    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.
    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.
    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.
    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. Respir Med. 2007;101:1350-1354.
    22.Lamas DJ et al. Am J Respir Crit Care Med. 2011;184:842-847.
    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.
    25.ATS Patient Information Series: Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;183:P1-P2. Available at https://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf. Accessed January 4, 2017.
    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.

     

    16