Introduction to IPF

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Introduction to IPF

There Are Over 150 Disorders that Comprise the Category of Interstitial Lung Disease

  • 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
The cause may also be unknown (idiopathic interstitial pneumonias)

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

IPF Is the Most Common Idiopathic Interstitial Pneumonia

Adapted from American Thoracic Society4; Travis WD et al.5
IPF, idiopathic pulmonary fibrosis.

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.
 

Overview of IPF

  • IPF is a progressive, fatal lung disease of unknown etiology6
  • Fibrosis is limited to the lung alveoli and interstitium6
  • A diagnosis of IPF requires6:
    • Exclusion of other known causes of ILD
    • The presence of a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) in patients not undergoing surgical lung biopsy
    • Specific combinations of HRCT patterns and, if necessary, surgical lung biopsy patterns
6. Raghu G et al. Am J Respir Crit Care Med. 2011;183:788-824.

IPF Is a Debilitating Lung Disease

Fatal lung disease affects adults >50 years of age,6 with a median survival of 3.8 years from diagnosis, generally the shortest of all ILDs7a
 
IPF is more common in men than women7
 
Most patients with IPF have a history of cigarette smoking6
 
The majority of patients experience a progressive decline, though the disease course is variable6
 
As fibrosis progresses, patients will become more breathless, less mobile, and less independent8

a
In the Medicare population.

6. Raghu G et al. Am J Respir Crit Care Med. 2011;183:788-824.
7. Raghu G et al. Lancet Respir Med. 2014;2:566-572.
8. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.

Risk Factors for IPF

  • Several environmental and genetic risk factors have been described despite its unknown etiology6
  • Genetic factors have been linked to both sporadic and familial IPF6
    • It is estimated that 5% of cases of IPF are familial6
    • A family history of IPF is a strong risk factor, increasing the odds up to 6-fold9
  • Familial and sporadic IPF are clinically, radiologically, and histologically identical6
It is hypothesized that these environmental and genetic risk factors contribute to epithelial cell injury and apoptosis during disease progression10,11
 
See About IPF: Risk Factors section for more detailed information.

6. Raghu G et al. Am J Respir Crit Care Med. 2011;183:788-824.
9. Garcia-Sancho C et al. Respir Med. 2011;105:1902-1907.
10. King TE Jr et al. Lancet. 2011;378:1949-1961.
11. Camelo A et al. Front Pharmacol. 2014;4:173.

The Clinical Course of IPF Is Variable and Unpredictable

Worsening of IPF may occur slowly or rapidly, and the disease course may be unexpectedly accelerated by acute exacerbations6,12,13

Acute exacerbations may occur at any time during the course of the disease6

See About IPF: Disease Course section for more detailed information.

6. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
12. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
13. Selman M et al. PLoS One. 2007;2(5):e482.

Common Symptoms Significantly Impact Quality of Life in Patients with IPF

Dyspnea
Cough
Fatigue
Depression


Dyspnea is universal in patients with IPF15 and is extremely distressing8

Chronic cough causes significant impairment in patients’ lives8

Fatigue/exhaustion occurs frequently,8 and is exacerbated by further muscle deconditioning15

Patients may experience fear, worry, anxiety, and panic related to their poor prognosis8,15

Dyspnea and physical function capacity are strong drivers of health-related quality of life14

8. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
14. Olson A et al. Patient Related Outcome Measures. 2016:7;29-35.
15. Lee JS et al. Curr Opin Pulm Med 2011;17(5):348-354.

Difficulty with Diagnosis Is Common in Patients With IPF

Difficulty with diagnosis is common, as the early symptoms of IPF are nonspecific and the disease itself is rare18,19
  • –Symptoms may be attributed to more common ailments such as bronchitis, asthma, COPD, emphysema, or heart disease19 
38% of patients report seeing 3 or more physicians before receiving a diagnosisof IPF19

>50% of patients are initially misdiagnosed19
 
1-2 year delay between onset of symptoms and diagnosis12,16-17

COPD, chronic obstructive pulmonary disease.

12. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
16. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
17. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644.
18. Cordier JF et al. Eur Respir J. 2013;42(4):916-923.
19. Collard HR et al. RespirMed. 2007;101:1350-1354.

The Diagnostic Process Can Take an Average of 1-2 Years

Bronchitis, asthma, COPD, emphysema, or heart disease are often considered first, as symptoms of IPF are common manifestations of these diseases19

12. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
16. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
17. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644.
19. Collard HR et al. Respir Med. 2007;101(6):1350-1354.
20. Lamas DJ et al. Am J Respir Crit Care Med. 2011;184:842-847.

There Are Several Diagnostic Tools for Evaluating Interstitial Lung Disease

Clinical Assessment
  • Comprehensive medical history6,21
  • Physical examination6
    • Chest auscultation6
  • Pulmonary function testing6,21:
    • FVC, FEV1, DLCO, lung volumes
  • 6-minute walk test (6MWT)6
  • Pulse oximetry22
  • Cardiac evaluation to rule out heart disease22
  • Serologic testing6
Radiological Assessment
  • Chest x-ray22
  • HRCT6
SurgicalAssessment
  • Bronchoscopy
  • Surgical lung biopsy (if necessary6)  
Multidisciplinary Meeting
  • Team-based approach to evaluating all evidence in order to reach a consensus diagnosis

See Diagnosing IPF: Diagnostic Criteria and Diagnostic Algorithm section for more detailed information.

FEV1, forced expiratory volume in 1 second.

6. Raghu G et al. Am J Respir Crit Care Med. 2011;183:788-824.
21. Wanger J et al. Eur Respir J. 2005;26:511-522.
22. 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.

Recommendations for Management of IPF

Recommendations include:
  • The majority of patients should begin pulmonaryrehabilitation6
  • Patients with clinically significant resting hypoxemia should begin long-term oxygen therapy6
  • Patients should be considered and evaluated for lung transplant in a timely manner6
  • All patients should be made aware of available medications and clinical trials for possible enrollment6
  • Patients should receive a yearly flu vaccine23
See Managing Patients: IPF Symptom Management section for more detailed information.

6. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
23. 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. 

Comprehensive Care for Patients with IPF Includes Several Patient-Centric Areas

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
See Managing Patients: Support section for more detailed information.

24. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Summary

  • IPF is a progressive, fatal disease that typically affects adults over 50 years of age25
  • Patients may experience periods of sudden respiratory decline termed acute exacerbations of IPF,25 which are associated with a high mortality rate26-28
  • Symptoms are nonspecific, but patients often present with chronic exertional dyspnea and a dry, nonproductive cough25
    • The average time from symptom onset to diagnosis is 1-2 years12,16-17
  • Diagnosis of IPF is difficult, often requiring discussion between pulmonologists, radiologists, and pathologists25,29
    • 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 biopsy25
  • Comprehensive care includes disease-focused, symptom-focused, and support-focused management24 
12. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
16. King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
17. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644.
24. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
25. Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816.
26. Kubo H et al. Chest. 2005;128:1475-1482.
27. Kim DS et al. Eur Respir J. 2006;27:143-150.
28. Daniels CE et al. Eur Respir J. 2008;32:170-174.
29. 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.Raghu G et al. Am J Respir Crit Care Med. 2011;183:788-824.
7.Raghu G et al. Lancet Respir Med. 2014;2:566-572.
8.Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
9.Garcia-Sancho C et al. Respir Med. 2011;105:1902-1907.
10.King TE Jr et al. Lancet. 2011;378:1949-1961.
11.Camelo A et al. Front Pharmacol. 2014;4:173.
12.Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
13.Selman M et al. PLoS One. 2007;2(5):e482.
14.Olson A et al. Patient Related Outcome Measures. 2016:7;29-35.
15.Lee JS et al. Curr Opin Pulm Med 2011;17(5):348-354.
16.King TE Jr et al. Am J Respir Crit Care Med. 2001;164(6):1025-1032.
17.Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644.
18.Cordier JF et al. Eur Respir J. 2013;42(4):916-923.
19.Collard HR et al. Respir Med. 2007;101:1350-1354.
20.Lamas DJ et al. Am J Respir Crit Care Med. 2011;184:842-847.
21.Wanger J et al. Eur Respir J. 2005;26:511-522.
22.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.
23.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.
24.Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
25.Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816.
26.Kubo H et al. Chest. 2005;128:1475-1482.
27.Kim DS et al. Eur Respir J. 2006;27:143-150.
28.Daniels CE et al. Eur Respir J. 2008;32:170-174.
29.Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910.