Идиопатический легочный фиброз: от диагностики к лечению

Шмелев Е.И. Идиопатический легочный фиброз: от диагностики к лечению. Consilium Medicum. 2018; 20 (3): 30–34. DOI: 10.26442/2075-1753_20.3.30-34

________________________________________________

Shmelev E.I. Idiopathic pulmonary fibrosis: from diagnosis to treatment. Consilium Medicum. 2018; 20 (3): 30–34. DOI: 10.26442/2075-1753_20.3.30-34

Читать PDF

Аннотация

Идиопатический легочный фиброз (ИЛФ) – тяжелая прогрессирующая болезнь с быстро (в течение нескольких лет) наступающим летальным исходом. Болезнь возникает во второй половине жизни, число больных возрастает. В 2011 г. опубликовано официальное соглашение Американского торакального общества и Европейского респираторного общества, представившее современную классификацию идиопатических интерстициальных пневмоний. Важной характеристикой течения заболевания является характер его прогрессирования. Выделяют по меньшей мере 3 варианта (фенотипа) течения ИЛФ. Особое место в течении ИЛФ занимают обострения. В Российском респираторном обществе созданы национальные рекомендации по диагностике и лечению больных ИЛФ с учетом международных соглашений, регистр больных ИЛФ.

Ключевые слова: идиопатический легочный фиброз, диагностика, лечение.

________________________________________________

Idiopathic pulmonary fibrosis (IРF) is a severe progressive disease with a rapidly (for several years) onset fatal outcome. The disease occurs in the second half of life, the number of patients increases. In 2011, an official agreement between The American thoracic society and the European respiratory society was published, presenting a modern classification of idiopathic interstitial pneumonia. An important characteristic of the course of the disease is the nature of its progression. Allocate at least 3 options (phenotype) of the IРF. A special place within the IРF is worsening. The Russian respiratory society has established National recommendations for the diagnosis and treatment of patients with IРF, taking into account international agreements, the register of patients with IРF.

Key words: idiopathic pulmonary fibrosis, diagnosis, treatment.

Полный текст

Материалы доступны только для специалистов сферы здравоохранения.
Чтобы посмотреть материал полностью Авторизуйтесь или зарегистрируйтесь.

Список литературы

2. Raghu G, Wycker D, Edelberg J et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 810–6.
3. Илькович М.М. Интерстициальные заболевания легких: рассуждения на актуальную тему. Болезни органов дыхания (Прил. к журн. Consilium Medicum). 2014; с. 4–9. / Ilkovich M.M. Interstitsialnye zabolevaniia legkikh: rassuzhdeniia na aktualnuiu temu. Respiratory Organs Diseases (Suppl. Consilium Medicum). 2014; p. 4–9. [in Russian]
4. Шмелев Е.И. Дифференциальная диагностика интерстициальных болезней легких. Consilium Medicum. 2003; 5 (4): 176–81. / Shmelev E.I. Differentsialnaia diagnostika interstitsialnykh boleznei legkikh. Consilium Medicum. 2003; 5 (4): 176–81. [in Russian]
5. Шмелев Е.И. Идиопатический легочный фиброз – современное состояние проблемы. Терапевт. арх. 2016; 88 (12): 103–8. / Shmelev E.I. Idiopaticheskii legochnyi fibroz – sovremennoe sostoianie problemy. Terapevt. arkh. 2016; 88 (12): 103–8. [in Russian]
6. Илькович М.М. Интерстициальные болезни легких. В кн.: Заболевания органов дыхания. СПб., 1998; с. 109–318. / Ilkovich M.M. Interstitsialnye bolezni legkikh. V kn.: Zabolevaniia organov dykhaniia. SPb., 1998; s. 109–318. [in Russian]
7. Sandbo N. Mechanismis of fibrosis in IPF. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 161–205.
8. Tiery JP, Sleeman JP. Complex networks orchestrate epithelial-mesenchymal transitions. Nat Rev Mol Cell Biol 2006; 7 (2): 131–42.
9. Cool CD, Groshong SD, Rai PR et al. Fibroblast foci are not discrete sites оf lung injury or repair: the fibroblast reticulum. Am J Respir Crit Care Med 2006; 174 (6): 654–8.
10. Marinkovic A, Mih JD, Park JA et al. Improved throughput traction microscopy reveals pivotal role for matrix stiffness in fibroblast contractility and TGF-b responsiveness. Am J Phisiol Lung Cell Mol Physiol 2012; 303 (3): 169–80.
11. Ryu JH, Moua T, Daniels CE et al. Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clin Proc 2014; 89: 1130–42.
12. Hewson T, McKeever TM, Gibson JE et al. Timing of onset of symptoms in people with idiopathic pulmonary fibrosis. Thorax 2017.
13. Tobin RW, Pope CE, Pellegrini CA et al. Increase prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158: 1804–8.
14. Raghu G, Yang ST, Spada C et al. Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series. Chest 2006; 129: 794–800.
15. Lettieri CJ, Nathan SD, Barnett S et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746–52.
16. Lancaster LH, Mason WR, Parnell JA et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 2009; 136: 772–8.
17. Brown AW, Shlobin OA, Weir N et al. Dynamic patient counseling: A novel concept in idiopathic pulmonary fibrosis. Chest 2012; 142: 1005–10.
18. Selman M, Carillo G, Estrada A et al. Accelerated variant of idiopathic pulmonary fibrosis. PloS One 2007; 2 (57): e482.
19. Trujiillo G, Meneghin A, Flahertty KR et al. TLR9 differentiates rapidly from slowly progressing forms of idiopathic pulmonary fibrosis. Sci Transl Med 2010; 2 (57): ra82.
20. Collard HR, Moore BB, Flaherty KR et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176 (7): 636–43.
21. Lee JS, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 349–58.
22. Wadhwa A, Flaherty KR. The keys to making a confident diagnosis of IPF. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 77–101.
23. Lynch DA et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6 (2): 138–53.
24. Wolters PJ et al. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? Lancet Respir Med 2018; 6 (2): 154–60.
25. Wells AU, Ward S. Pulmonary Function tests in Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 103–21.
26. Gibson GJ. Clinical tests of respiratory function. London: Chappel and Hall, 1996; p. 233–4.
27. Welss AU, Desai SR, Rubens MB et al. Idiopathic Pulmonary Fibrosis: a composite phisiologic index derived from disease extend observed on computer tomography. Am J Respir Crit Care Med 2003; 167: 962–9.
28. Hunninghake GW, Zimmerman MB, Schwartz DA et al. Utility of lung biopsy for diagnosis of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2001; 164 (2): 193–6.
29. Utz JP, Ryu JH, Douglas WW et al. High short-term mortality following lung biopssy for usual interstitial pneumonia. Eur Resp J 2001; 17 (2): 175–9.
30. Lettieri CJ, Veerappan GR, Helman DL et al. Outcomes and safety of surgical lung biopsy for interstitial lung disease. Chest 2005; 127 (5): 1600–5.
31. Canver CC, Mencer RMJr. The role of open lung biopsy in early and late survival of ventilator-depends patients with diffuse idiopathic lung disease. J Cardiovasc Surg (Torino) 1994; 35 (2): 151–5.
32. Mushiroda T, Wattanapokayakit S, Takahashi A et al. A genome-wide association study identifies an association of a common variant in TERT with susceptibility to idiopathic pulmonary fibrosis. J Med Genet 2008; 45: 654–6.
33. Seibold MA, Wise AL, Speer MC et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011; 364: 1503–12.
34. King TE et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083–92.
35. Raghu G, Bram R, Yuan Z et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis: Executive Summary. Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015; 192 (2): 237–48.
36. Cores J et al. Safety and Efficacy of Allogeneic Lung Spheroid Cells in a Mismatched Rat Model of Pulmonary Fibrosis. Stem Cells Transl Med 2017; 6 (10): 1905–16.
37. Herrmann FE et al. Olodaterol shows anti-fibrotic efficacy in in vitro and in vivo models of pulmonary fibrosis. Br J Pharmacol 2017; 174 (21): 3848–64.
38. Mailleux AA, Crestani B. Licence to kill senescent cells in idiopathic pulmonary fibrosis? Eur Respir J 2017; 50 (2).
39. Xin LL, Jiang M, Zhang G, Gong JN. Zhongguo Zhong Yao Za Zhi 2016; 41 (20): 3859–65.
40. Holland AE, Swigris DO. The role of pulmonary Rehabilitation and supplemental oxygen therapy in the treatment of patients with Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 337–47.
41. Lamas DJ, Lederer DJ. Lung transplantation for Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 363–77.

________________________________________________

1. Raghu G, Collard HR, Egan JJ 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. Raghu G, Wycker D, Edelberg J et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 810–6.
3. Ilkovich M.M. Interstitsialnye zabolevaniia legkikh: rassuzhdeniia na aktualnuiu temu. Respiratory Organs Diseases (Suppl. Consilium Medicum). 2014; p. 4–9. [in Russian]
4. Shmelev E.I. Differentsialnaia diagnostika interstitsialnykh boleznei legkikh. Consilium Medicum. 2003; 5 (4): 176–81. [in Russian]
5. Shmelev E.I. Idiopaticheskii legochnyi fibroz – sovremennoe sostoianie problemy. Terapevt. arkh. 2016; 88 (12): 103–8. [in Russian]
6. Ilkovich M.M. Interstitsialnye bolezni legkikh. V kn.: Zabolevaniia organov dykhaniia. SPb., 1998; s. 109–318. [in Russian]
7. Sandbo N. Mechanismis of fibrosis in IPF. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 161–205.
8. Tiery JP, Sleeman JP. Complex networks orchestrate epithelial-mesenchymal transitions. Nat Rev Mol Cell Biol 2006; 7 (2): 131–42.
9. Cool CD, Groshong SD, Rai PR et al. Fibroblast foci are not discrete sites оf lung injury or repair: the fibroblast reticulum. Am J Respir Crit Care Med 2006; 174 (6): 654–8.
10. Marinkovic A, Mih JD, Park JA et al. Improved throughput traction microscopy reveals pivotal role for matrix stiffness in fibroblast contractility and TGF-b responsiveness. Am J Phisiol Lung Cell Mol Physiol 2012; 303 (3): 169–80.
11. Ryu JH, Moua T, Daniels CE et al. Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clin Proc 2014; 89: 1130–42.
12. Hewson T, McKeever TM, Gibson JE et al. Timing of onset of symptoms in people with idiopathic pulmonary fibrosis. Thorax 2017.
13. Tobin RW, Pope CE, Pellegrini CA et al. Increase prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158: 1804–8.
14. Raghu G, Yang ST, Spada C et al. Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series. Chest 2006; 129: 794–800.
15. Lettieri CJ, Nathan SD, Barnett S et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746–52.
16. Lancaster LH, Mason WR, Parnell JA et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 2009; 136: 772–8.
17. Brown AW, Shlobin OA, Weir N et al. Dynamic patient counseling: A novel concept in idiopathic pulmonary fibrosis. Chest 2012; 142: 1005–10.
18. Selman M, Carillo G, Estrada A et al. Accelerated variant of idiopathic pulmonary fibrosis. PloS One 2007; 2 (57): e482.
19. Trujiillo G, Meneghin A, Flahertty KR et al. TLR9 differentiates rapidly from slowly progressing forms of idiopathic pulmonary fibrosis. Sci Transl Med 2010; 2 (57): ra82.
20. Collard HR, Moore BB, Flaherty KR et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176 (7): 636–43.
21. Lee JS, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 349–58.
22. Wadhwa A, Flaherty KR. The keys to making a confident diagnosis of IPF. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 77–101.
23. Lynch DA et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6 (2): 138–53.
24. Wolters PJ et al. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? Lancet Respir Med 2018; 6 (2): 154–60.
25. Wells AU, Ward S. Pulmonary Function tests in Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 103–21.
26. Gibson GJ. Clinical tests of respiratory function. London: Chappel and Hall, 1996; p. 233–4.
27. Welss AU, Desai SR, Rubens MB et al. Idiopathic Pulmonary Fibrosis: a composite phisiologic index derived from disease extend observed on computer tomography. Am J Respir Crit Care Med 2003; 167: 962–9.
28. Hunninghake GW, Zimmerman MB, Schwartz DA et al. Utility of lung biopsy for diagnosis of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2001; 164 (2): 193–6.
29. Utz JP, Ryu JH, Douglas WW et al. High short-term mortality following lung biopssy for usual interstitial pneumonia. Eur Resp J 2001; 17 (2): 175–9.
30. Lettieri CJ, Veerappan GR, Helman DL et al. Outcomes and safety of surgical lung biopsy for interstitial lung disease. Chest 2005; 127 (5): 1600–5.
31. Canver CC, Mencer RMJr. The role of open lung biopsy in early and late survival of ventilator-depends patients with diffuse idiopathic lung disease. J Cardiovasc Surg (Torino) 1994; 35 (2): 151–5.
32. Mushiroda T, Wattanapokayakit S, Takahashi A et al. A genome-wide association study identifies an association of a common variant in TERT with susceptibility to idiopathic pulmonary fibrosis. J Med Genet 2008; 45: 654–6.
33. Seibold MA, Wise AL, Speer MC et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011; 364: 1503–12.
34. King TE et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083–92.
35. Raghu G, Bram R, Yuan Z et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis: Executive Summary. Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015; 192 (2): 237–48.
36. Cores J et al. Safety and Efficacy of Allogeneic Lung Spheroid Cells in a Mismatched Rat Model of Pulmonary Fibrosis. Stem Cells Transl Med 2017; 6 (10): 1905–16.
37. Herrmann FE et al. Olodaterol shows anti-fibrotic efficacy in in vitro and in vivo models of pulmonary fibrosis. Br J Pharmacol 2017; 174 (21): 3848–64.
38. Mailleux AA, Crestani B. Licence to kill senescent cells in idiopathic pulmonary fibrosis? Eur Respir J 2017; 50 (2).
39. Xin LL, Jiang M, Zhang G, Gong JN. Zhongguo Zhong Yao Za Zhi 2016; 41 (20): 3859–65.
40. Holland AE, Swigris DO. The role of pulmonary Rehabilitation and supplemental oxygen therapy in the treatment of patients with Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 337–47.
41. Lamas DJ, Lederer DJ. Lung transplantation for Idiopathic Pulmonary Fibrosis. In: Idiopathic Pulmonary Fibrosis: A Comprehensive Clinical Guide. New York: Springer Science, 2014; p. 363–77.

Авторы

Е.И.Шмелев

ФГБНУ «Центральный научно-исследовательский институт туберкулеза». 107564, Россия, Москва, Яузская аллея, д. 2

*eishmelev@mail.ru

________________________________________________

E.I.Shmelev

Central Research Institute of Tuberculosis. 107564, Russian Federation, Moscow, Yauzskaia alleyia, d. 2
*eishmelev@mail.ru

Назад к списку

Цель портала OmniDoctor – предоставление профессиональной информации врачам, провизорам и фармацевтам.