Idiopathic Pulmonary Fibrosis Treatment & Management

  • Author: Amanda Godfrey, MD; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: May 23, 2012
 

Medical Care

The goal of any disease management strategy should include assessment and treatment of comorbid medical conditions. Common comorbid medical conditions found in patients with idiopathic pulmonary fibrosis (IPF) include chronic obstructive pulmonary disease, obstructive sleep apnea, gastroesophageal reflux disease, and coronary artery disease. Therefore, if any of these comorbid illnesses are present, they should be managed according to current practice guidelines.

Given the high prevalence of gastroesophageal reflux (GER) in patients with IPF, a retrospective study was conducted to investigate the relationship of reflux-related variables and survival time in patients with IPF. Of the 204 included patients, 34% reported symptoms of GER, 45% had a history of GER disease, 47% reported use of medications for GER, and 5% of patients had previously undergone Nissen fundoplication. On adjusted analysis, the use of GER medications was associated with a longer survival time. Additionally, patients taking GER medications had a lower fibrosis score on HRCT.[33]

Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit and offered pharmacologic therapy if needed.

Patients with hypoxemia (PaO2 < 55 mmHg or oxygen saturation as measured using pulse oximetry [SpO2] < 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion.

Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.

See Medication for a discussion of the various drugs, experimental and otherwise, used in the treatment of idiopathic pulmonary fibrosis.

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Surgical Care

Lung transplantation for idiopathic pulmonary fibrosis has been shown to confer a survival benefit over medical therapy. In May 2005, the lung allocation score (LAS) was implemented, which dramatically changed lung allocation from a system based purely on waiting time to an algorithm based on survival probability on the waiting list and after lung transplantation.[34] Therefore, the LAS is used to prioritize patients based on the difference between post-transplant 1-year survival and pre-transplant urgency. Consequent to the use of LAS, idiopathic pulmonary fibrosis has now replaced chronic obstructive pulmonary disease as the most common indication for lung transplantation in the United States.[35]

Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred for lung transplantation evaluation, regardless of the vital capacity.[36] After a patient is referred for transplantation evaluation, the appropriate timing to list a patient on the lung transplantation list needs to be determined. Guidelines for listing a patient with idiopathic pulmonary fibrosis include a diffusion capacity of carbon monoxide (DLCO) less than 39% predicted, a 10% or greater decrement in forced vital capacity during 6 months of follow-up, a decrease in pulse oximetry below 88% during a 6-minute walk test (6MWT), or honeycombing on high-resolution computed tomography (HRCT) imaging (fibrosis score >2).[36]

A 2009 retrospective review of the United Network for Organ Sharing data to identify lung transplant recipients with idiopathic pulmonary fibrosis between 2005 and 2007 examined risk for 30-day, 90-day, and 1-year mortality for single lung transplant versus bilateral lung transplant. Data were examined across levels of the LAS (quartile 1, quartile 2, quartile 3, and quartile 4).

Patients in LAS quartile 4 were defined as high risk. A clear inverse relationship between wait-list times and LAS was seen, with a higher LAS score associated with shorter wait-list times.[35] Patients in the LAS quartile 4 had a 7.1% lower cumulative survival at 1 year when compared with patients in quartiles 1 to 3. Just over 21% more patients received bilateral lung transplantation in the highest LAS quartile than in the lowest LAS quartile. In the high-risk quartile, bilateral lung transplantation was associated with a 14.4% decrease in mortality 1 year after lung transplantation.[35] However, this study is limited by the retrospective nature and the need to see if these trends persist at 3 years and 5 years. The reported 5-year survival rates after lung transplantation in idiopathic pulmonary fibrosis are estimated at 50-56%.[1]

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Consultations

Any patient suspected of having idiopathic pulmonary fibrosis, interstitial lung disease, or any another idiopathic interstitial pneumonia should be referred to a pulmonologist for further evaluation and management. Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred for lung transplantation evaluation, regardless of the vital capacity.[36]

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Diet

Any patient with idiopathic pulmonary fibrosis who is overweight should be encouraged to meet with a nutritionist and make dietary changes to achieve ideal body weight. Maintaining adequate nutritional intake is important for quality of life in patients with idiopathic pulmonary fibrosis.

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Activity

Improving quality of life is an important goal in disease management. Deconditioning and subsequent functional impairment is a common problem in patients with idiopathic pulmonary fibrosis and negatively impacts quality of life. Two controlled trials of pulmonary rehabilitation in idiopathic pulmonary fibrosis have demonstrated an improvement in walk distance and symptoms or quality of life.[1] Therefore, patients with idiopathic pulmonary fibrosis should be evaluated for pulmonary rehabilitation and encouraged to participate in regular exercise to maintain a maximal degree of musculoskeletal conditioning.[3]

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Contributor Information and Disclosures
Author

Amanda Godfrey, MD  Fellow, Pulmonary and Critical Care Medicine, Henry Ford Hospital

Amanda 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, and 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; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP  Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

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, and Sigma Xi

Disclosure: See below for list of all activities None None

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

Disclosure: Medscape Salary Employment

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

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

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

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.

References

  1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med. Mar 15 2011;183(6):788-824. [Medline].

  2. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. Jan 15 2002;165(2):277-304. [Medline].

  3. Frankel SK, Schwarz MI. Update in idiopathic pulmonary fibrosis. Curr Opin Pulm Med. Sep 2009;15(5):463-9. [Medline].

  4. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Feb 15 2011;183(4):431-40. [Medline].

  5. Kim DS, Collard HR, King TE Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. Jun 2006;3(4):285-92. [Medline]. [Full Text].

  6. Misumi S, Lynch DA. Idiopathic pulmonary fibrosis/usual interstitial pneumonia: imaging diagnosis, spectrum of abnormalities, and temporal progression. Proc Am Thorac Soc. Jun 2006;3(4):307-14. [Medline].

  7. Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc. Jun 2006;3(4):322-9. [Medline].

  8. Verma S, Slutsky AS. Idiopathic pulmonary fibrosis--new insights. N Engl J Med. Mar 29 2007;356(13):1370-2. [Medline].

  9. Harari S, Caminati A. IPF: new insight on pathogenesis and treatment. Allergy. May 2010;65(5):537-53. [Medline].

  10. Thannickal VJ, Horowitz JC. Evolving concepts of apoptosis in idiopathic pulmonary fibrosis. Proc Am Thorac Soc. Jun 2006;3(4):350-6. [Medline]. [Full Text].

  11. Maher TM, Evans IC, Bottoms SE, Mercer PF, Thorley AJ, Nicholson AG. Diminished prostaglandin E2 contributes to the apoptosis paradox in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Jul 1 2010;182(1):73-82. [Medline].

  12. Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. Mar 29 2007;356(13):1317-26. [Medline].

  13. Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. Apr 21 2011;364(16):1503-12. [Medline].

  14. Wang XM, Zhang Y, Kim HP, et al. Caveolin-1: a critical regulator of lung fibrosis in idiopathic pulmonary fibrosis. J Exp Med. Dec 25 2006;203(13):2895-906. [Medline]. [Full Text].

  15. Fernandez Perez ER, Daniels CE, Schroeder DR, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest. Jan 2010;137(1):129-37. [Medline]. [Full Text].

  16. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, Brown KK. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med. Aug 1 2007;176(3):277-84. [Medline].

  17. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Oct 1 2006;174(7):810-6. [Medline].

  18. Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med. Jan 15 2008;177(2):190-4. [Medline].

  19. Lancaster LH, Mason WR, Parnell JA, et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest. Sep 2009;136(3):772-8. [Medline]. [Full Text].

  20. Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. Sep 2007;132(3):998-1006. [Medline].

  21. Fishman A, Elias J, Fishman J, Grippi M, Senior R, Pack A. Idiopathic Pulmonary Fibrosis. In: Fishman AP. Fishman's Pulmonary Diseases and Disorders. Vol 1. 4th ed. The McGraw-Hill Companies, Inc.; 2008:1143-60.

  22. Lawson WE, Loyd JE. The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease?. Proc Am Thorac Soc. Jun 2006;3(4):345-9. [Medline]. [Full Text].

  23. Wootton SC, Kim DS, Kondoh Y, et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Jun 15 2011;183(12):1698-702. [Medline]. [Full Text].

  24. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. Feb 2003;58(2):143-8. [Medline]. [Full Text].

  25. Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. Proc Am Thorac Soc. Jun 2006;3(4):315-21. [Medline]. [Full Text].

  26. Tzilas V, Koti A, Papandrinopoulou D, Tsoukalas G. Prognostic factors in idiopathic pulmonary fibrosis. Am J Med Sci. Dec 2009;338(6):481-5. [Medline].

  27. du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med. Dec 15 2011;184(12):1382-9. [Medline].

  28. Flaherty KR, Andrei AC, Murray S, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med. Oct 1 2006;174(7):803-9. [Medline]. [Full Text].

  29. Swigris JJ, Swick J, Wamboldt FS, et al. Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis. Chest. Sep 2009;136(3):841-8. [Medline]. [Full Text].

  30. Bois RM, Weycker D, Albera C, et al. Six-minute-walk test in idiopathic pulmonary fibrosis: test validation and minimal clinically important difference. Am J Respir Crit Care Med. May 1 2011;183(9):1231-7. [Medline].

  31. Ohshimo S, Bonella F, Cui A, et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Jun 1 2009;179(11):1043-7. [Medline].

  32. Kinder BW, Brown KK, Schwarz MI, Ix JH, Kervitsky A, King TE Jr. Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest. Jan 2008;133(1):226-32. [Medline].

  33. Lee JS, Ryu JH, Elicker BM, Lydell CP, Jones KD, Wolters PJ, et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Dec 15 2011;184(12):1390-4. [Medline].

  34. Kozower BD, Meyers BF, Smith MA, et al. The impact of the lung allocation score on short-term transplantation outcomes: a multicenter study. J Thorac Cardiovasc Surg. Jan 2008;135(1):166-71. [Medline].

  35. Weiss ES, Allen JG, Merlo CA, Conte JV, Shah AS. Survival after single versus bilateral lung transplantation for high-risk patients with pulmonary fibrosis. Ann Thorac Surg. Nov 2009;88(5):1616-25; discussion 1625-6. [Medline].

  36. Orens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. Jul 2006;25(7):745-55. [Medline].

  37. Behr J, Kolb M, Cox G. Treating IPF--all or nothing? A PRO-CON debate. Respirology. Nov 2009;14(8):1072-81. [Medline].

  38. Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. Nov 24 2005;353(21):2229-42. [Medline].

  39. Prednisone, Azathioprine, and N-Acetylcysteine for Pulmonary Fibrosis. N Engl J Med. May 20 2012;[Medline].

  40. National Heart, Lung, and Blood Institute. Clinical Alert: Commonly Used Three-Drug Regimen for Pulmonary Fibrosis Found Harmful. NIH Stops One Treatment Arm of Trial; Other Two Treatments to Continue. US National Library of Medicine: National Institutes of Health; October 21, 2011. [Full Text].

  41. Raghu G, Brown KK, Bradford WZ, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. Jan 8 2004;350(2):125-33. [Medline].

  42. Raghu G, Brown KK, Costabel U, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med. Nov 1 2008;178(9):948-55. [Medline].

  43. King TE Jr, Albera C, Bradford WZ, et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet. Jul 18 2009;374(9685):222-8. [Medline].

  44. King TE Jr, Behr J, Brown KK, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Jan 1 2008;177(1):75-81. [Medline].

  45. The Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. Aug 12 2010;363(7):620-8. [Medline].

  46. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR. Imatinib treatment for idiopathic pulmonary fibrosis: Randomized placebo-controlled trial results. Am J Respir Crit Care Med. Mar 15 2010;181(6):604-10. [Medline].

  47. Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 365:1079-1087.

  48. Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. May 1 2005;171(9):1040-7. [Medline].

  49. Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. Apr 2010;35(4):821-9. [Medline].

  50. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. May 21 2011;377(9779):1760-9. [Medline].

  51. Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. Chest. Nov 2007;132(5):1652-8. [Medline].

  52. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. Feb 2011;37(2):356-63. [Medline].

  53. du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Aug 15 2011;184(4):459-66. [Medline].

  54. Ley B, Ryerson CJ, Vittinghoff E, et al. A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis. Annals of Internal Medicine. 2012/05;156:684-691.

  55. Fang A, Studer S, Kawut SM, Ahya VN, Lee J, Wille K. Elevated Pulmonary Artery Pressure Is a Risk Factor for Primary Graft Dysfunction Following Lung Transplantation for Idiopathic Pulmonary Fibrosis. Chest. Apr 2011;139(4):782-787. [Medline].

  56. Kinder BW, Brown KK, McCormack FX, et al. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest. Jun 2009;135(6):1557-63. [Medline]. [Full Text].

  57. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. Feb 2000;161(2 Pt 1):646-64. [Medline].

  58. Patel AS, Siegert RJ, Brignall K, Gordon P, Steer S, Desai SR. The development and validation of the King's Brief Interstitial Lung Disease (K-BILD) health status questionnaire. Thorax. May 3 2012;[Medline].

 
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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.
PredictorPoints
SexFemale0
Male1
Age (years)≥600
61-651
>652
FVC (% predicted)>750
50-751
< 502
DLCO (% predicted)>550
36-551
≤352
Cannot perform3
Table 2. Staging and mortality risk for IPF.
StageIIIIII
Points0-34-56-8
Mortality
1-year5.616.239.2
2-year10.929.962.1
3-year16.342.176.8
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