, Volume 190, Issue 4, pp 373–380 | Cite as

Exacerbations in Idiopathic Pulmonary Fibrosis Triggered by Pulmonary and Nonpulmonary Surgery: A Case Series and Comprehensive Review of the Literature

  • Abhijeet Ghatol
  • A. Parker Ruhl
  • Sonye K. Danoff



Acute exacerbations (AE) of idiopathic pulmonary fibrosis (IPF) are well recognized in the progression of this uniformly fatal disease. Surgical lung biopsy and lung resection may initiate these acute events leading to a rapid deterioration and permanent decline in lung function. Our aim is to discuss the role of pulmonary and nonpulmonary surgery as a precipitating factor and to review the literature on the nature, course, and outcomes of acute exacerbations in the context of surgical interventions.


This study consisted of a retrospective case series of patients at the Johns Hopkins Hospital who experienced acute exacerbation following a surgical procedure. Patients included in the case series suffered from aggravation of dyspnea within 1 month after surgical intervention, with new infiltrates on imaging. There was no other more likely cause after diagnostic evaluation. A comprehensive review of the current literature pertaining to AEs of IPF in the context of a surgical intervention was performed.


In a series of four patients from Johns Hopkins Hospital with AE in IPF, two of three patients who underwent video-assisted thoracoscopic surgery (VATS) lung biopsy had a fatal outcome. The fourth patient survived an AE after a total knee replacement but had a fatal outcome after a subsequent coronary artery bypass graft surgery. We found no report in the literature of AE in an IPF patient who underwent nonpulmonary surgery.


Acute exacerbations of IPF can occur postoperatively after both pulmonary and nonpulmonary surgery and are associated with a high mortality rate. As a next step, a prospective multicenter clinical study of patients with IPF undergoing both pulmonary and nonpulmonary surgeries would allow the identification of perioperative risk factors in the development of AE of IPF.


Acute exacerbation Idiopathic pulmonary fibrosis Surgery Circulating fibrocytes 



Acute exacerbation


Idiopathic pulmonary fibrosis


Video assisted thoracoscopic surgery


Intensive care unit


Acute lung injury/acute respiratory distress syndrome


Circulating fibrocytes


Total knee replacement


  1. 1.
    American Thoracic Society (2000) 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 161:646–664Google Scholar
  2. 2.
    American Thoracic Society; European Respiratory Society (2002) 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 165:277–304Google Scholar
  3. 3.
    Watanabe A, Kawaharada N, Higami T (2011) Postoperative acute exacerbation of IPF after lung resection for primary lung cancer. Pulm Med 2011:960316PubMedGoogle Scholar
  4. 4.
    Kondoh Y, Taniguchi H, Kitaichi M, Yokoi T, Johkoh T, Oishi T et al (2006) Acute exacerbation of interstitial pneumonia following surgical lung biopsy. Respir Med 100:1753–1759PubMedCrossRefGoogle Scholar
  5. 5.
    Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T (2006) Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J 27:143–150PubMedCrossRefGoogle Scholar
  6. 6.
    Sigurdsson MI, Isaksson HJ, Gudmundsson G, Gudbjartsson T (2009) Diagnostic surgical lung biopsies for suspected interstitial lung diseases: a retrospective study. Ann Thorac Surg 88(1):227–232PubMedCrossRefGoogle Scholar
  7. 7.
    Park JH, Kim DK, Kim DS, Koh Y, Lee SD, Kim WS, Kim WD, Park SI (2007) Mortality and risk factors for surgical lung biopsy in patients with idiopathic interstitial pneumonia. Eur J Cardiothorac Surg 31:1115–1119PubMedCrossRefGoogle Scholar
  8. 8.
    Kreider ME, Hansen-Flaschen J, Ahmad NN, Rossman MD, Kaiser LR, Kucharczuk JC, Shrager JB (2007) Complications of video-assisted thoracoscopic lung biopsy in patients with interstitial lung disease. Ann Thorac Surg 83(3):1140–1144PubMedCrossRefGoogle Scholar
  9. 9.
    Utz JP, Ryu JH, Douglas WW et al (2001) High short-term mortality following lung biopsy for usual interstitial pneumonia. Eur Respir J 17:175–179PubMedCrossRefGoogle Scholar
  10. 10.
    Tiitto L, Heiskanen U, Bloigu R et al (2005) Thoracoscopic lung biopsy is a safe procedure in diagnosing usual interstitial pneumonia. Chest 128:2375–2380PubMedCrossRefGoogle Scholar
  11. 11.
    Lettieri CJ, Veerappan GR, Helman DL, Mulligan CR, Shorr AF (2005) Outcomes and safety of surgical lung biopsy for interstitial lung disease. Chest 127:1600–1605PubMedCrossRefGoogle Scholar
  12. 12.
    Suzuki H, Sekine Y, Yoshida S, Suzuki M et al (2011) Risk of acute exacerbation of interstitial pneumonia after pulmonary resection for lung cancer in patients with idiopathic pulmonary fibrosis based on preoperative high-resolution computed tomography. Surg Today 41:914–921PubMedCrossRefGoogle Scholar
  13. 13.
    Shintani Y, Ohta M, Iwasaki T, Ikeda N, Tomita E, Kawahara K, Ohno Y (2010) Predictive factors for postoperative acute exacerbation of interstitial pneumonia combined with lung cancer. Gen Thorac Cardiovasc Surg 58(4):182–185PubMedCrossRefGoogle Scholar
  14. 14.
    Kushibe K, Kawaguchi T, Takahama M, Kimura M, Tojo T, Taniguchi S (2007) Operative indications for lung cancer with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg 55(8):505–508CrossRefGoogle Scholar
  15. 15.
    Watanabe A, Higami T, Ohori S, Koyanagi T, Nakashima S, Mawatari T (2008) Is lung cancer resection indicated in patients with idiopathic pulmonary fibrosis? J Thorac Cardiovasc Surg 136(5):1357–1363PubMedCrossRefGoogle Scholar
  16. 16.
    Kawasaki H, Nagai K, Yoshida J, Nishimura M, Nishiwaki Y (2002) Postoperative morbidity, mortality, and survival in lung cancer associated with idiopathic pulmonary fibrosis. J Surg Oncol 81(1):33–37PubMedCrossRefGoogle Scholar
  17. 17.
    Saydain G, Islam A, Afessa B et al (2002) Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med 166:839–842PubMedCrossRefGoogle Scholar
  18. 18.
    Al-Hameed FM, Sharma S (2004) Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis. Can Respir J 11:117–122PubMedGoogle Scholar
  19. 19.
    Bucala R, Spiegel LA, Chesney J, Hogan M, Cerami A (1994) Circulating fibrocytes define a new leukocyte subpopulation that mediates tissue repair. Mol Med 1(1):71–81PubMedGoogle Scholar
  20. 20.
    Strieter RM, Keeley EC, Burdick MD, Mehrad B (2009) The role of circulating mesenchymal progenitor cells, fibrocytes, in promoting pulmonary fibrosis. Trans Am Clin Climatol Assoc 120:49–59PubMedGoogle Scholar
  21. 21.
    Quan TE, Cowper S, Wu SP, Bockenstedt LK, Bucala R (2004) Circulating fibrocytes: collagen-secreting cells of the peripheral blood. Int J Biochem Cell Biol 36(4):598–606PubMedCrossRefGoogle Scholar
  22. 22.
    Phillips RJ, Burdick MD, Hong K, Lutz MA, Murray LA, Xue YY, Belperio JA, Keane MP, Strieter RM (2004) Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis. J Clin Invest 114(3):438–446PubMedGoogle Scholar
  23. 23.
    Schmidt M, Sun G, Stacey MA, Mori L, Mattoli S (2003) Identification of circulating fibrocytes as precursors of bronchial myofibroblasts in asthma. J Immunol 171(1):380–389PubMedGoogle Scholar
  24. 24.
    Chauhan H, Abraham A, Phillips JR, Pringle JH, Walker RA, Jones JL (2003) There is more than one kind of myofibroblast: analysis of CD34 expression in benign, in situ, and invasive breast lesions. J Clin Pathol 56(4):271–276PubMedCrossRefGoogle Scholar
  25. 25.
    Moeller A, Gilpin SE, Ask K, Cox G, Cook D, Gauldie J, Margetts PJ, Farkas L, Dobranowski J, Boylan C, O’Byrne PM, Strieter RM, Kolb M (2009) Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 179(7):588–594PubMedCrossRefGoogle Scholar
  26. 26.
    Kubo H, Nakayama K, Yanai M, Suzuki T, Yamaya M, Watanabe M, Sasaki H (2005) Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 128(3):1475–1482PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Abhijeet Ghatol
    • 1
  • A. Parker Ruhl
    • 2
    • 3
  • Sonye K. Danoff
    • 2
  1. 1.Department of Pulmonary and Critical Care MedicineCedars Sinai Medical CenterLos AngelesUSA
  2. 2.Division of Pulmonary and Critical Care MedicineJohns Hopkins UniversityBaltimoreUSA
  3. 3.Critical Care Medicine DepartmentNational Institutes of HealthBethesdaUSA

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