Modern approaches to acute exacerbation of idiopathic pulmonary fibrosis


DOI: https://dx.doi.org/10.18565/therapy.2019.6.127-138

Avdeev S.N.

1) I.M. Sechenov First Moscow State medical University of the Ministry of Healthcare of Russia; 2) Pulmonology research Institute of FMBA of Russia, Moscow
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute, clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormalities such as diffuse, bilateral ground-glass opacification and/or consolidation, and the absence of other obvious clinical causes like fluid overload, left heart failure, or pulmonary embolism, etc. AE-IPF are subcategorized as «triggered» (where specific causes are identified, for example, infections, surgery procedures, drug toxicity, etc.) or «idiopathic» (where no specific causes are identified).
In randomized trials, the annual incidence of AE-IPF is about 8%, in retrospective studies it reaches 19%. Severe forms of IPF are an important risk factor for the development of AE-IPF. Intrahospital mortality of AE-IPF is more than 50%, and the average survival of patients with AE-IPF is 1–4 months.
Currently, there remain no proven, effective therapies for AE-IPF. In real clinical practice patients with AE-IPF still receive high doses of systemic corticosteroids and antibiotics. Antifibrotic therapy can reduce the risk of exacerbations, it has been shown that therapy with nintedanib leads to a reduction in the number of confirmed/suspected AE-IPF by 68%. It is necessary to further study the potential methods of prevention and therapy of AE-IPF in future clinical trials.

Literature



  1. Raghu G., Collard H.R., Egan J.J. et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011; 183: 788–824.

  2. Чучалин А.Г., Авдеев С.Н., Айсанов З.Р., Белевский А.С., Демура С.А., Илькович М.М., Коган Е.А., Самсонова М.В., Сперанская А.А., Тюрин И.Е., Черняев А.Л., Черняк Б.А., Черняк А.В., Шмелев Е.И. Диагностика и лечение идиопатического легочного фиброза Федеральные клинические рекомендации. Пульмонология. 2016; 26(4): 399–419. https://doi.org/10.18093/0869-0189-2016-26-4-399-419.

  3. Авдеев С.Н. Идиопатический легочный фиброз: новая парадигма. Терапевтический архив. 2017; 89(1): 112–122.

  4. Bjoraker J.A., Ryu J.H., Edwin M.K. et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998; 157: 199–203.

  5. Ley B., Ryerson C.J., Vittinghoff E. et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012; 156: 684–91.

  6. Martinez F.J., Safrin S., Weycker D. et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005; 142: 963–67.

  7. King T.E. Jr, Albera C., Bradford W.Z. et al. All-cause mortality rate in patients with idiopathic pulmonary fibrosis. Implications for the design and execution of clinical trials. Am J Respir Crit Care Med. 2014; 189: 825–31.

  8. Kondoh Y., Taniguchi H., Kawabata Y. et al. Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathological findings in three cases. Chest. 1993; 103: 1808–12.

  9. Авдеев С.Н., Черняев А.Л., Самсонова М.В., Мержоева З.М. Обострение идиопатического легочного фиброза. Пульмонология. 2006; 4: 123–127.

  10. Ambrosini V, Cancellieri A, Chilosi M. et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J. 2003; 22: 821–26.

  11. Collard H.R., Moore B.B., Flaherty K.R. et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007; 176: 636–43.

  12. Leuschner G., Behr J. Acute exacerbation in interstitial lung disease. Frontiers in Medicine 2017; 4: article 176.

  13. Collard H.R., Ryerson C.J., Corte T.J., Jenkins G., Kondoh Y., Lederer D.J. et al. Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med. 2016; 194: 265–75. doi:10.1164/ rccm.201604-0801CI

  14. Kim D.S., Park J.H., Park B.K., Lee J.S., Nicholson A.G., Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006; 27:143–50. doi:10.1183/09031936.06.00114004

  15. Song J.W., Hong S.B., Lim C.M., Koh Y., Kim D.S. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011; 37:356–63. doi:10.1183/09031936.00159709

  16. Akira M., Hamada H., Sakatani M., Kobayashi C., Nishioka M., Yamamoto S. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol. 1997; 168: 79–83. doi:10.2214/ ajr.168.1.8976924

  17. Kishaba T., Tamaki H., Shimaoka Y., Fukuyama H., Yamashiro S. Staging of acute exacerbation in patients with idiopathic pulmonary fibrosis. Lung 2014; 192: 141–49. doi: 10.1007/s00408-013-9530-0

  18. Akira M., Kozuka T., Yamamoto S., Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008; 178: 372–78. doi:10.1164/rccm.200709-1365OC

  19. Fujimoto K., Taniguchi H., Johkoh T. et al. Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 2012; 22: 83–92. doi: 10.1007/ s00330-011-2211-6

  20. Usui Y., Kaga A., Sakai F. et al. A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia. BMJ Open. 2013; 3: e002971. doi: 10.1136/ bmjopen-2013-002971

  21. Parambil J.G., Myers J.L., Ryu J.H. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis under-going surgical lung biopsy. Chest. 2005; 128: 3310–15. doi: 10.1378/chest. 128.5.3310

  22. Черняев А.Л., Самсонова М.В. Диффузное альвеолярное повреждение: этиология, патогенез и патологическая анатомия. Пульмонология 2005; 4: 65–69.

  23. Churg A., Muller N.L., Silva C.I., Wright J.L. Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias. Am J Surg Pathol. 2007; 31: 277–84. doi: 10.1097/01.pas.0000213341.70852.9d

  24. Dallari R., Foglia M., Paci M., Cavazza A. Acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J. 2004; 23: 792. doi: 10.1183/09031936.04.00 004404

  25. Collard H.R., Calfee C.S., Wolters P.J. et al. Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2010; 299: L3–7. doi: 10.1152/ ajplung.90637.2008

  26. Ohshimo S., Ishikawa N., Horimasu Y. et al. Baseline KL-6 predicts increased risk for acute exacerbation of idiopathic pulmonary fibrosis. Respir Med. 2014; 108: 1031–39. doi: 10.1016/j. rmed.2014.04.009

  27. Kishaba T., Shimaoka Y., Fukuyama H. et al. A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema. BMJ Open. 2012; 2: e000988. doi: 10.1136/bmjopen-2012-000988

  28. Arcadu A., Moua T. Bronchoscopy assessment of acute respiratory failure in interstitial lung disease. Respirology. 2017; 22: 352–59.

  29. Takei R., Arita M., Kumagai S. et al. Impact of lymphocyte differential count>15% in BALF on the mortality of patients with acute exacerbation of chronic fibrosing idiopathic interstitial pneumonia. BMC Pulm Med. 2017; 17: 67. doi: 10.1186/s12890-017-0412-8

  30. Ryerson C.J., Cottin V., Brown K.K., Collard H.R. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015; 46: 512–20. doi: 10.1183/13993003.00419-2015

  31. Collard H.R., Yow E., Richeldi L., Anstrom K.J., Glazer C. IPFnet Investigators. Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials. Respir Res. 2013; 14: 73. doi: 10.1186/1465-9921-14-73

  32. Atkins C.P., Loke Y.K., Wilson A.M. Outcomes in idiopathic pulmonary fibrosis: a meta-analysis from placebo controlled trials. Respir Med. 2014; 108: 376–87. doi: 10.1016/j.rmed.2013.11.007

  33. Richeldi L., du Bois R.M., Raghu G. et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370: 2071–82. doi: 10.1056/NEJMoa1402584

  34. Kondoh Y., Taniguchi H., Katsuta T. et al. Risk factors of acute exacerbation of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2010; 27: 103–10.

  35. Johannson K.A., Vittinghoff E., Lee K. et al. Acute exacerbation of idiopathic pulmonary fibrosis associated with air pollution exposure. Eur Respir J. 2014; 43: 1124–31. doi: 10.1183/09031936.00122213

  36. Kondoh Y., Taniguchi H., Ebina M. et al. Risk factors for acute exacerbation of idiopathic pulmonary fibrosis – extended analysis of pirfenidone trial in Japan. Respir Investig. 2015; 53: 271–78. doi: 10.1016/j. resinv.2015.04.005

  37. Sakamoto S., Homma S., Mun M., Fujii T., Kurosaki A., Yoshimura K. Acute exacerbation of idiopathic interstitial pneumonia following lung surgery in 3 of 68 consecutive patients: a retrospective study. Intern Med. 2011; 50: 77–85. doi:10.2169/internalmedicine.50.3390

  38. Samejima J., Tajiri M., Ogura T. et al. Thoracoscopic lung biopsy in 285 patients with diffuse pulmonary disease. Asian Cardiovasc Thorac Ann. 2015; 23: 191–97. doi: 10.1177/0218492314550724

  39. Rotolo N., Imperatori A., Dominioni L. et al. Efficacy and safety of surgical lung biopsy for interstitial disease. Experience of 161 consecutive patients from a single institution in Italy. Sarcoidosis Vasc Diffuse Lung Dis. 2015; 32: 251–58.

  40. Bando M., Ohno S., Hosono T. et al. Risk of acute exacerbation after video-assisted thoracoscopic lung biopsy for interstitial lung disease. J Bronchology Interv Pulmonol. 2009; 16: 229–35. doi: 10.1097/ LBR.0b013e3181b767cc

  41. Taniguchi H., Ebina M., Kondoh Y. et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010; 35: 821–29. doi:10.1183/09031936.00005209

  42. Costabel U., Inoue Y., Richeldi L. et al. Efficacy of nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016; 193: 178–85. doi:10.1164/rccm.201503-0562OC

  43. Kuhn C. III, Boldt J., King T.E. Jr, Crouch E., Vartio T., McDonald J.A. An immunohistochemical study of architectural remodeling and connective tis-sue synthesis in pulmonary fibrosis. Am Rev Respir Dis. 1989; 140: 1693–1703. doi: 10.1164/ajrccm/140.6.1693

  44. Konishi K., Gibson K.F., Lindell K.O. et al. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009; 180: 167–75. doi: 10.1164/rccm. 200810-1596OC

  45. Mukae H., Iiboshi H., Nakazato M. et al. Raised plasma concentrations of alpha-defensins in patients with idiopathic pulmonary fibrosis. Thorax 2002; 57: 623–28. doi: 10.1136/thorax.57.7.623

  46. van Wetering S., Sterk P.J., Rabe K.F., Hiemstra P.S. Defensins: key players or bystanders in infection, injury, and repair in the lung? J Allergy Clin Immunol. 1999; 104: 1131–38. doi:10.1016/S0091-6749(99)70004-7

  47. Ganz T., Lehrer R.I. Defensins. Curr Opin Immunol. 1994; 6: 584–89. doi: 10.1016/0952-7915(94)90145-7

  48. Moeller A., Gilpin S.E., Ask K. et al. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009; 179: 588–94. doi: 10.1164/ rccm.200810-1534OC

  49. Bucala R., Spiegel L.A., Chesney J., Hogan M., Cerami A. Circulating fibrocytes define a new leukocyte subpopulation that mediates tissue repair. Mol Med. 1994; 1: 71–81.

  50. Gomperts B.N., Strieter R.M. Fibrocytes in lung disease. J Leukoc Biol. 2007; 82: 449–56. doi:10.1189/jlb.0906587

  51. Mantovani A., Sica A., Sozzani S., Allavena P., Vecchi A., Locati M. The chemokine system in diverse forms of macrophage activation and polarization. Trends Immunol. 2004; 25: 677–86. doi: 10.1016/j.it.2004.09.015

  52. Schupp J.C., Binder H., Jager B. et al. Macrophage activation in acute exacerbation of idiopathic pulmonary fibrosis. PLoS One. 2015; 10: e0116775. doi: 10.1371/journal.pone.0116775

  53. Ushiki A., Yamazaki Y., Hama M. Yasuo M., Hanaoka M., Kubo K. Viral infections in patients with an acute exacerbation of idiopathic interstitial pneumonia. Respir Investig. 2014; 52: 65–70. doi: 10.1016/j.resinv.2013.07.005

  54. Wootton S.C., Kim D.S., Kondoh Y. et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011; 183: 1698–702. doi: 10.1164/rccm.201010-1752OC

  55. Huie T.J., Olson A.L., Cosgrove G.P. et al. A detailed evaluation of acute respiratory decline in patients with fibrotic lung disease: aetiology and outcomes. Respirology. 2010; 15: 909–17. doi: 10.1111/j. 1440-1843.2010.01774.x

  56. Molyneaux P.L., Cox M.J., Wells A.U. et al. Changes in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis. Respir Res. 2017; 18: 29. doi: 10.1186/s12931-017-0511-3

  57. Simon-Blancal V., Freynet O., Nunes H. et al. Acute exacerbation of idiopathic pulmonary fibrosis: outcome and prognostic factors. Respiration 2012; 83: 28–35. doi: 10.1159/000329891

  58. Petrosyan F., Culver D.A., Reddy A.J. Role of bronchoalveolar lavage in the diagnosis of acute exacerbations of idiopathic pulmonary fibrosis: a retrospective study. BMC Pulm Med. 2015; 15: 70. doi: 10.1186/s12890-015-0066-3

  59. Lee J.S., Collard H.R., Anstrom K.J. et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013; 1: 369–76. doi: 10.1016/S2213-2600(13)70105-X

  60. Lee J.S., Song J.W., Wolters P.J. et al. Bronchoalveolar lavage pepsin in acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J. 2012; 39: 352–58. doi: 10.1183/09031936.00050911

  61. Mizuno Y., Iwata H., Shirahashi K. et al. The importance of intraoperative fluid balance for the prevention of postoperative acute exacerbation of idiopathic pulmonary fibrosis after pulmonary resection for primary lung cancer. Eur J Cardiothorac Surg. 2012; 41: e161–65. doi: 10.1093/ejcts/ezs147

  62. Suzuki H., Sekine Y., Yoshida S. et al. 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. 2011; 41: 914–21. doi: 10.1007/s00595-010-4384-z

  63. Watanabe A., Higami T., Ohori S., Koyanagi T., Nakashima S., Mawatari T. Is lung cancer resection indicated in patients with idiopathic pulmonary fibrosis? J Thorac Cardiovasc Surg. 2008; 136: 1357–63, 1363.e1–2. doi: 10.1016/j. jtcvs.2008.07.016

  64. Park J.S., Kim H.K., Kim K., Kim J., Shim Y.M., Choi Y.S. Prediction of acute pulmonary complications after resection of lung cancer in patients with preexisting interstitial lung disease. Thorac Cardiovasc Surg. 2011; 59: 148–52. doi: 10.1055/s-0030-1250644

  65. Ghatol A., Ruhl A.P., Danoff S.K. Exacerbations in idiopathic pulmonary fibrosis triggered by pulmonary and nonpulmonary surgery: a case series and comprehensive review of the literature. Lung. 2012; 190: 373–80. doi: 10.1007/ s00408-012-9389-5

  66. Choi S.M., Lee J., Park Y.S. et al. Postoperative pul-monary complications after surgery in patients with interstitial lung disease. Respiration. 2014; 87: 287–93. doi: 10.1159/000357046

  67. Hiwatari N., Shimura S., Takishima T., Shirato K. Bronchoalveolar lavage as a possible cause of acute exacerbation in idiopathic pulmonary fibrosis patients. Tohoku J Exp Med. 1994; 174: 379–86. doi: 10.1620/tjem.174.379

  68. Sakamoto K., Taniguchi H., Kondoh Y. et al. Acute exacerbation of IPF following diagnostic bronchoalveolar lavage procedures. Respir Med. 2012; 106: 436–42. doi: 10.1016/j.rmed.2011.11.006

  69. Tomic R., Cortes-Puentes G.A., Murugan P., Joo Kim H., Amin K., Dincer H.E. Acute exacerbation of interstitial lung disease after cryobiopsy. J Bronchology Interv Pulmonol. 2017; 24: 319–22. doi: 10.1097/LBR.0000000000000369

  70. Casoni G.L., Tomassetti S., Cavazza A. et al. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases. PLoS One. 2014; 9: e86716. doi: 10.1371/journal.pone.0086716

  71. Selman M., Carrillo G., Estrada A. et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007; 2: e482. doi: 10.1371/journal. pone.0000482

  72. Russell A.M., Adamali H., Molyneaux P.L. et al. Daily home spirometry: an effective tool for detecting progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016; 194: 989–97. doi: 10.1164/rccm.201511-2152OC

  73. Judge E.P., Fabre A., Adamali H.I., Egan J.J. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J. 2012; 40: 93–100. doi: 10.1183/09031936.00115511

  74. Ohshimo S., Ishikawa N., Horimasu Y. et al. Baseline KL-6 predicts increased risk for acute exacerbation of idiopathic pulmonary fibrosis. Respir Med 2014; 108: 1031–39. doi: 10.1016/j. rmed.2014.04.009

  75. Horita N., Akahane M., Okada Y. et al. Tacrolimus and steroid treatment for acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2011; 50: 189–95. doi: 10.2169/internalmedicine.50.4327

  76. Kakiuchi S., Hanibuchi M., Tezuka T. et al. Analysis of acute exacerbation of interstitial lung disease associated with chemotherapy in patients with lung cancer: a feasibility of S-1. Respir Investig. 2017; 55: 145–52. doi: 10.1016/j.resinv.2016.10.008

  77. Takeda A., Enomoto T., Sanuki N. et al. Acute exacerbation of subclinical idiopathic pulmonary fibrosis triggered by hypofractionated stereotactic body radiotherapy in a patient with primary lung cancer and slightly focal honeycombing. Radiat Med. 2008; 26: 504–07. doi: 10.1007/s11604-008-0261-8

  78. Parambil J.G., Myers J.L., Aubry M.C., Ryu J.H. Causes and prognosis of diffuse alveolar damage diagnosed on surgical lung biopsy. Chest. 2007; 132: 50–57. doi: 10.1378/chest.07-0104

  79. Jeon K., Chung M.P., Lee K.S. et al. Prognostic factors and causes of death in Korean patients with idiopathic pulmonary fibrosis. Respir Med. 2006; 100: 451–57. doi: 10.1016/j.rmed.2005.06.013

  80. Seo Y., Abe S., Kurahara M. et al. Beneficial effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2006; 45: 1033–38. doi: 10.2169/internalmedicine.45.6018

  81. Tachikawa R., Tomii K., Ueda H. et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration. 2012; 83: 20–27. doi: 10.1159/000329893

  82. Olson A.L., Huie T.J., Groshong S.D. et al. Acute exacerbations of fibrotic hypersensitivity pneumonitis: a case series. Chest. 2008; 134: 844–50. doi:10.1378/chest.08-0428

  83. Suda T., Kaida Y., Nakamura Y. et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 2009; 103: 846–53. doi: 10.1016/j. rmed.2008.12.019

  84. Miyazaki Y., Tateishi T., Akashi T., Ohtani Y., Inase N., Yoshizawa Y. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest. 2008; 134: 1265–70. doi: 10.1378/ chest.08-0866

  85. Kahloon R.A., Xue J., Bhargava A. et al. Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am J Respir Crit Care Med. 2013; 187: 768–75. doi: 10.1164/rccm.201203-0506OC

  86. Morell F., Esser D., Lim J. et al. Treatment patterns, resource use and costs of idiopathic pulmonary fibrosis in Spain – results of a Delphi Panel. BMC Pulm Med. 2016; 16: 7.

  87. Yu Y.F., Wu N., Chuang C.C. et al. Patterns and economic burden of hospitalizations and exacerbations among patients diagnosed with idiopathic pulmonary fibrosis. J Manag Care Spec Pharm. 2016; 22: 414–23.

  88. Bozso S., Sidhu S., Garg M., Freed D.H., Nagendran J. Canada’s longest experience with extracorporeal membrane oxygenation as a bridge to lung transplantation: a case report. Transplant Proc. 2015; 47: 186–89. doi: 10.1016/j. transproceed.2014.10.039

  89. Rush B., Wiskar K., Berger L., Griesdale D. The use of mechanical ventilation in patients with idiopathic pulmonary fibrosis in the United States: a nationwide retrospective cohort analysis. Respir Med. 2016; 111: 72–76. doi: 10.1016/j. rmed.2015.12.005

  90. Inase N., Sawada M., Ohtani Y. et al. Cyclosporin A followed by the treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroid. Intern Med 2003; 42: 565–70. doi:10.2169/internalmedicine.42.565

  91. Homma S., Sakamoto S., Kawabata M. et al. Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia. Intern Med. 2005; 44: 1144–50. doi: 10.2169/internalmedicine. 44.1144

  92. Sakamoto S., Homma S., Miyamoto A., Kurosaki A., Fujii T., Yoshimura K. Cyclosporin A in the treatment of acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2010; 49: 109–15. doi: 10.2169/internalmedicine.49.2359

  93. Donahoe M., Valentine V.G., Chien N. et al. Autoantibody-targeted treatments for acute exacerbations of idiopathic pulmonary fibrosis. PLoS One. 2015; 10: e0127771. doi: 10.1371/journal.pone.0127771

  94. Abe S., Azuma A., Mukae H. et al. Polymyxin B-immobilized fiber column (PMX) treatment for idiopathic pulmonary fibrosis with acute exacerbation: a multicenter retrospective analysis. Intern Med. 2012; 51: 1487–91. doi: 10.2169/internalmedicine.51.6965

  95. Oishi K., Aoe K., Mimura Y. et al. Survival from an acute exacerbation of idiopathic pulmonary fibrosis with or without direct hemoperfusion with a polymyxin B-immobilized fiber column: a retrospective analysis. Intern Med. 2016; 55: 3551–59. doi: 10.2169/ internalmedicine.55.6056

  96. Oishi K., Mimura-Kimura Y., Miyasho T. et al. Association between cytokine removal by polymyxin B hemoperfusion and improved pulmonary oxygenation in patients with acute exacerbation of idiopathic pulmonary fibrosis. Cytokine. 2013; 61: 84–89. doi: 10.1016/j. cyto.2012.08.032

  97. Isshiki T., Sakamoto S., Kinoshita A., Sugino K., Kurosaki A., Homma S. Recombinant human soluble thrombomodulin treatment for acute exacerbation of idiopathic pulmonary fibrosis: a retrospective study. Respiration. 2015; 89: 201–07. doi: 10.1159/000369828

  98. Tsushima K., Yamaguchi K., Kono Y. et al. Thrombomodulin for acute exacerbations of idiopathic pulmonary fibrosis: a proof of concept study. Pulm Pharmacol Ther. 2014; 29: 233–40. doi: 10.1016/j.pupt.2014.04.008

  99. Kataoka K., Taniguchi H., Kondoh Y. et al. Recombinant human thrombomodulin in acute exacerbation of idiopathic pulmonary fibrosis. Chest. 2015; 148: 436–43. doi: 10.1378/chest.14-2746

  100. Hayakawa S., Matsuzawa Y., Irie T., Rikitake H., Okada N., Suzuki Y. Efficacy of recombinant human soluble thrombomodulin for the treatment of acute exacerbation of idiopathic pulmonary fibrosis: a single arm, non-randomized prospective clinical trial. Multidiscip Respir Med. 2016; 11: 38. doi: 10.1186/ s40248-016-0074-z

  101. Ding J., Chen Z., Feng K. Procalcitonin-guided antibiotic use in acute exacerbations of idiopathic pulmonary fibrosis. Int J Med Sci. 2013; 10: 903–07. doi: 10.7150/ijms.4972

  102. Yokoyama T., Kondoh Y., Taniguchi H. et al. Noninvasive ventilation in acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2010; 49: 1509–14. doi: 10.2169/ internalmedicine.49.3222

  103. Papiris S.A., Kagouridis K., Kolilekas L. et al. Survival in idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach. BMC Pulm Med. 2015; 15: 162. doi: 10.1186/s12890-015-0146-4

  104. 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. 2005; 171: 1040–47. doi: 10.1164/ rccm.200404-571OC

  105. King T.E. Jr, Bradford W.Z., Castro-Bernardini S. et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370: 2083–92. doi: 10.1056/ NEJMoa1402582

  106. Noble P.W., Albera C., Bradford W.Z. et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011; 377: 1760–69. doi: 10.1016/ S0140-6736(11)60405-4

  107. Ley B., Swigris J., Day B.M. et al. Pirfenidone reduces respiratory-related hospitalizations in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2017; 196(6): 756–61. doi: 10.1164/ rccm.201701-0091OC

  108. Iwata T., Yoshida S., Nagato K. et al. Experience with perioperative pirfenidone for lung cancer surgery in patients with idiopathic pulmonary fibrosis. Surg Today. 2015; 45: 1263–70. doi: 10.1007/s00595-014-1071-5

  109. Richeldi L., Costabel U., Selman M. et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011; 365: 1079–87. doi: 10.1056/NEJMoa1103690

  110. Collard H.R., Richeldi L., Kim D.S. et al. Acute exacerbations in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Eur Respir J. 2017; 49: 1601339 [https://doi.org/10.1183/13993003.01339-2016].

  111. Idiopathic Pulmonary Fibrosis Clinical Research Network, Martinez F.J., de Andrade J.A., Anstrom K.J., King T.E. Jr., Raghu G. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014; 370: 2093–2101. doi: 10.1056/NEJMoa1401739

  112. Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman D.A., Schwarz M., Anstrom K.J., Collard H.R., Flaherty K.R. et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010; 363: 620–28. doi:10.1056/NEJMoa1002110

  113. King T.E. Jr., Brown K.K., Raghu G. et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011; 184: 92–99. doi: 10.1164/ rccm.201011-1874OC

  114. King T.E. Jr, Albera C., Bradford W.Z. et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet. 2009; 374: 222–28. doi: 10.1016/S0140- 6736(09)60551-1

  115. Noth I., Anstrom K.J., Calvert S.B. et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2012; 186: 88–95. doi: 10.1164/ rccm.201202-0314OC

  116. Raghu G., Behr J., Brown K.K. et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013; 158: 641–49. doi: 10.7326/0003-4819- 158-9-201305070-00003

  117. Daniels C.E., Lasky J.A., Limper A.H. et al. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med. 2010; 181: 604–10. doi: 10.1164/rccm.200906-0964OC

  118. Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G., Anstrom K.J., King T.E. Jr., Lasky J.A., Martinez F.J. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012; 366: 1968–77. doi: 10.1056/NEJMoa1113354

  119. Lamas D.J., Kawut S.M., Bagiella E., Philip N., Arcasoy S.M., Lederer D.J. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med. 2011; 184: 842–47. doi: 10.1164/rccm.201104-0668OC


About the Autors


Sergey N. Avdeev, MD, professor, corresponding member of RAS, head of the Department of pulmonology of I.M. Sechenov First Moscow State medical University of the Ministry of Healthcare of Russia, head of Clinical Department of Pulmonology research Institute of FMBA of Russia. Address: 105077, Moscow, 28 Orekhovy boulevard. Tel./fax: +7 (495) 395-63-93. E-mail: serg_avdeev@list.ru. https://orcid.org/0000-0002-5999-2150


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