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Клинические характеристики идиопатического легочного фиброза и гиперчувствительного пневмонита в реальной клинической практике
Клинические характеристики идиопатического легочного фиброза и гиперчувствительного пневмонита в реальной клинической практике
Суворова О.А., Трушенко Н.В., Лавгинова Б.Б., Левина Ю.А., Мержоева З.М., Авдеев С.Н. Клинические характеристики идиопатического легочного фиброза и гиперчувствительного пневмонита в реальной клинической практике. Consilium Medicum. 2025;27(3):179–186. DOI: 10.26442/20751753.2025.3.203265
© ООО «КОНСИЛИУМ МЕДИКУМ», 2025 г.
© ООО «КОНСИЛИУМ МЕДИКУМ», 2025 г.
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Аннотация
Обоснование. Идиопатический легочный фиброз (ИЛФ) и гиперчувствительный пневмонит (ГП) – интерстициальные заболевания легких со схожими симптомами (одышкой и кашлем), которые определяют тяжесть состояния пациентов и считаются прогностическими маркерами.
Цель. Сравнение клиническо-функциональных характеристик и их динамики у пациентов с ИЛФ и различными фенотипами ГП, оценка взаимосвязи симптомов с данными объективных методов исследования.
Материалы и методы. В продольное наблюдательное исследование включены 153 пациента [48 – с ИЛФ, 105 – ГП, из них 72 – фибротический (фГП), включая 32 с обычной интерстициальной пневмонией (ОИП)]. Проанализированы демографические, анамнестические и функциональные параметры (спирометрия, бодиплетизмография, диффузионная способность легких по монооксиду углерода), оценены одышка по шкале mMRC и кашель по Визуальной аналоговой шкале и опроснику LCQ, результаты 6-минутного теста ходьбы (6МТХ), изменения по высокоразрешающей компьютерной томографии (ВРКТ) и эхокардиографии (дисфункция правых отделов сердца) исходно и через 12 мес. Статистический анализ включал непараметрические методы сравнения двух групп, корреляционный анализ по методу Спирмена, множественную линейную регрессию для оценки влияния параметров на одышку и кашель.
Результаты. Пациенты с фГП и фГП+ОИП по сравнению с пациентами с ИЛФ значимо не отличались по выраженности одышки (mMRC), интенсивности кашля (Визуальная аналоговая шкала) и функциональным показателям. Летальность и прогрессирование в группах ИЛФ и фГП, а также фГП+ОИП сопоставимы. Выраженность одышки по шкале mMRC коррелировала с баллами GAP (Gender – Age – Physiology), дистанцией 6МТХ, SpO2 в начале и в конце 6МТХ, наличием фиброза по ВРКТ, функциональными показателями (диффузионная способность легких по монооксиду углерода, Composite Physiologic Index и др.), индексом коморбидности Чарльсона. Регрессионный анализ показал, что влияние на выраженность одышки оказывали SpO2 в конце 6МТХ и наличие легочного фиброза. Кашель коррелировал с наличием фиброза, одышкой (mMRC) и функциональными показателями (форсированная жизненная емкость легких, объем форсированного выдоха за 1 с, CPI). Наиболее значимыми факторами, определяющими интенсивность кашля, были объем форсированного выдоха за 1 с и наличие легочного фиброза.
Заключение. Пациенты с фГП, особенно с паттерном ОИП, по тяжести функциональных нарушений и клинических проявлений сопоставимы с пациентами с ИЛФ. Одышка и кашель тесно связаны с десатурацией при нагрузке, функциональными показателями и наличием легочного фиброза по ВРКТ, что следует учитывать при оценке клинической картины ГП и ИЛФ.
Ключевые слова: интерстициальные заболевания легких, гиперчувстительный пневмонит, идиопатический легочный фиброз, одышка, кашель
Aim. To compare clinical and functional characteristics and their dynamics in patients with IPF and different HP phenotypes, and to assess the relationship between symptoms and objective test results.
Materials and methods. A longitudinal observational study included 153 patients (48 with IPF, 105 with HP, including 72 fibrotic HP [fHP], of whom 32 had a UIP pattern). Demographic, anamnestic, and functional parameters (spirometry, body plethysmography, DLco) were analyzed. Dyspnea (mMRC scale), cough (VAS and LCQ questionnaire), 6MWT results, HRCT changes, and echocardiographic signs of right heart dysfunction were assessed at baseline and after 12 months. Statistical analysis included nonparametric tests, Spearman correlation, and multiple linear regression to evaluate the impact of parameters on dyspnea and cough.
Results. Patients with fHP and fHP+UIP did not significantly differ from IPF patients in dyspnea severity (mMRC), cough intensity (VAS), or functional parameters. Mortality and disease progression were comparable between IPF, fHP, and fHP+UIP groups. Dyspnea severity correlated with GAP scores, 6MWT distance, SpO2 at baseline and end of 6MWT, presence of fibrosis on HRCT, functional parameters (DLco, CPI, etc.), and Charlson Comorbidity Index. Regression analysis showed that end-exercise SpO2 and pulmonary fibrosis had the greatest impact on dyspnea. Cough correlated with fibrosis, dyspnea (mMRC), and functional parameters (FVC, FEV1, CPI). FEV1 and pulmonary fibrosis were the most significant predictors of cough severity.
Conclusion. Patients with fHP, particularly those with a UIP pattern, have comparable functional impairment and clinical manifestations to IPF patients. Dyspnea and cough are closely associated with exertional desaturation, functional parameters, and pulmonary fibrosis on HRCT, which should be considered when evaluating HP and IPF.
Keywords: interstitial lung diseases, hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, dyspnea, cough
Цель. Сравнение клиническо-функциональных характеристик и их динамики у пациентов с ИЛФ и различными фенотипами ГП, оценка взаимосвязи симптомов с данными объективных методов исследования.
Материалы и методы. В продольное наблюдательное исследование включены 153 пациента [48 – с ИЛФ, 105 – ГП, из них 72 – фибротический (фГП), включая 32 с обычной интерстициальной пневмонией (ОИП)]. Проанализированы демографические, анамнестические и функциональные параметры (спирометрия, бодиплетизмография, диффузионная способность легких по монооксиду углерода), оценены одышка по шкале mMRC и кашель по Визуальной аналоговой шкале и опроснику LCQ, результаты 6-минутного теста ходьбы (6МТХ), изменения по высокоразрешающей компьютерной томографии (ВРКТ) и эхокардиографии (дисфункция правых отделов сердца) исходно и через 12 мес. Статистический анализ включал непараметрические методы сравнения двух групп, корреляционный анализ по методу Спирмена, множественную линейную регрессию для оценки влияния параметров на одышку и кашель.
Результаты. Пациенты с фГП и фГП+ОИП по сравнению с пациентами с ИЛФ значимо не отличались по выраженности одышки (mMRC), интенсивности кашля (Визуальная аналоговая шкала) и функциональным показателям. Летальность и прогрессирование в группах ИЛФ и фГП, а также фГП+ОИП сопоставимы. Выраженность одышки по шкале mMRC коррелировала с баллами GAP (Gender – Age – Physiology), дистанцией 6МТХ, SpO2 в начале и в конце 6МТХ, наличием фиброза по ВРКТ, функциональными показателями (диффузионная способность легких по монооксиду углерода, Composite Physiologic Index и др.), индексом коморбидности Чарльсона. Регрессионный анализ показал, что влияние на выраженность одышки оказывали SpO2 в конце 6МТХ и наличие легочного фиброза. Кашель коррелировал с наличием фиброза, одышкой (mMRC) и функциональными показателями (форсированная жизненная емкость легких, объем форсированного выдоха за 1 с, CPI). Наиболее значимыми факторами, определяющими интенсивность кашля, были объем форсированного выдоха за 1 с и наличие легочного фиброза.
Заключение. Пациенты с фГП, особенно с паттерном ОИП, по тяжести функциональных нарушений и клинических проявлений сопоставимы с пациентами с ИЛФ. Одышка и кашель тесно связаны с десатурацией при нагрузке, функциональными показателями и наличием легочного фиброза по ВРКТ, что следует учитывать при оценке клинической картины ГП и ИЛФ.
Ключевые слова: интерстициальные заболевания легких, гиперчувстительный пневмонит, идиопатический легочный фиброз, одышка, кашель
________________________________________________
Aim. To compare clinical and functional characteristics and their dynamics in patients with IPF and different HP phenotypes, and to assess the relationship between symptoms and objective test results.
Materials and methods. A longitudinal observational study included 153 patients (48 with IPF, 105 with HP, including 72 fibrotic HP [fHP], of whom 32 had a UIP pattern). Demographic, anamnestic, and functional parameters (spirometry, body plethysmography, DLco) were analyzed. Dyspnea (mMRC scale), cough (VAS and LCQ questionnaire), 6MWT results, HRCT changes, and echocardiographic signs of right heart dysfunction were assessed at baseline and after 12 months. Statistical analysis included nonparametric tests, Spearman correlation, and multiple linear regression to evaluate the impact of parameters on dyspnea and cough.
Results. Patients with fHP and fHP+UIP did not significantly differ from IPF patients in dyspnea severity (mMRC), cough intensity (VAS), or functional parameters. Mortality and disease progression were comparable between IPF, fHP, and fHP+UIP groups. Dyspnea severity correlated with GAP scores, 6MWT distance, SpO2 at baseline and end of 6MWT, presence of fibrosis on HRCT, functional parameters (DLco, CPI, etc.), and Charlson Comorbidity Index. Regression analysis showed that end-exercise SpO2 and pulmonary fibrosis had the greatest impact on dyspnea. Cough correlated with fibrosis, dyspnea (mMRC), and functional parameters (FVC, FEV1, CPI). FEV1 and pulmonary fibrosis were the most significant predictors of cough severity.
Conclusion. Patients with fHP, particularly those with a UIP pattern, have comparable functional impairment and clinical manifestations to IPF patients. Dyspnea and cough are closely associated with exertional desaturation, functional parameters, and pulmonary fibrosis on HRCT, which should be considered when evaluating HP and IPF.
Keywords: interstitial lung diseases, hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, dyspnea, cough
Полный текст
Список литературы
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2. O'Brien EC, Hellkamp AS, Neely ML, et al. IPF-PRO Registry investigators. Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry. Chest. 2020;157(5):1188-98. DOI:10.1016/j.chest.2019.11.042
3. Fernández Pérez ER, Swigris JJ, Forssén AV, et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest. 2013;144(5):1644-51. DOI:10.1378/chest.12-2685
4. Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69. DOI:10.1164/rccm.202005-2032ST
5. Liao YW, Chen YM, Liu MC, et al. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024;29(1):69. DOI:10.1186/s40001-024-01644-7
6. Lee J, White E, Freiheit E, et al. Pulmonary Fibrosis Foundation. Cough-Specific Quality of Life Predicts Disease Progression Among Patients With Interstitial Lung Disease: Data From the Pulmonary Fibrosis Foundation Patient Registry. Chest. 2022;162(3):603-13. DOI:10.1016/j.chest.2022.03.025
7. Khor YH, Johannson KA, Marcoux V, et al. CARE-PF Investigators. Epidemiology and Prognostic Significance of Cough in Fibrotic Interstitial Lung Disease. Am J Respir Crit Care Med. 2024;210(8):1035-44. DOI:10.1164/rccm.202311-2101OC
8. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, et al. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis. PLoS One. 2016;11(3):e0151425. DOI:10.1371/journal.pone.0151425
9. Seixas E, Ferreira M, Serra P, et al. Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort. Afr J Thorac Crit Care Med. 2022;28(4). DOI:10.7196/AJTCCM.2022.v28i4.250
10. Adams TN, Batra K, Kypreos M, Glazer CS. Impact of radiographic honeycombing on transplant free survival and efficacy of immunosuppression in fibrotic hypersensitivity pneumonitis. BMC Pulm Med. 2023;23(1):224. DOI:10.1186/s12890-023-02523-3
11. Avdeev SN, Aisanov ZR, Vizel AA, et al. Federal clinical guidelines on diagnosis and treatment of hypersensitivity pneumonitis. Pulmonology. 2025;35(1):16-41 (in Russian). DOI:10.18093/0869-0189-2025-35-1-16-41
12. Suvorova OA, Trushenko NV, Samsonova MV, et al. Hypersensitivity pneumonitis: the current state of the problem. Russian Journal of Occupational Health and Industrial Ecology. 2024;63(6):408-19 (in Russian). DOI:10.31089/1026-9428-2024-64-6-408-419
13. Raghu G, Remy-Jardin M, Myers JL, et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. DOI:10.1164/rccm.201807-1255ST
14. Fletcher CM, Elmes PC, Fairbairn AS, Wood CH. The significance of respiratory symptoms and the diagnosis of chronic bronchitis in a working population. Br Med J. 1959;2:256-66. DOI:10.1136/bmj.2.5147.257
15. Birring SS, Prudon B, Carr AJ, et al. Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax. 2003;58(4):339-43. DOI:10.1136/thorax.58.4.339
16. Charlson ME, Pompei P, Ales KL, MacKenzie CR. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis. 1987;40(5):373-83. DOI:10.1016/0021-9681(87)90171-8
17. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-91. DOI:10.7326/0003-4819-156-10-201205150-00004
18. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. DOI:10.1183/13993003.01499-2021
19. Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167(7):962-9. DOI:10.1164/rccm.2111053
20. Alberti ML, Malet Ruiz JM, Fernández ME, et al. Comparative survival analysis between idiopathic pulmonary fibrosis and chronic hypersensitivity pneumonitis. Pulmonology. 2020;26(1):3-9. DOI:10.1016/j.pulmoe.2019.08.007
21. Maher TM, Brown KK, Kreuter M, et al. INBUILD trial investigators. Effects of nintedanib by inclusion criteria for progression of interstitial lung disease. Eur Respir J. 2022;59(2):2004587. DOI:10.1183/13993003.04587-2020
22. Juliá-Serdá G, Navarro-Esteva J, Doreste-Salgado L, et al. Survival and Lung Function Changes in Hypersensitivity Pneumonitis According to Radiological Phenotypes Compared With Idiopathic Pulmonary Fibrosis. Cureus. 2024;16(3):e57307. DOI:10.7759/cureus.57307
23. Petnak T, Cheungpasitporn W, Thongprayoon C, et al. Phenotypic subtypes of fibrotic hypersensitivity pneumonitis identified by machine learning consensus clustering analysis. Respir Res. 2024;25(1):41. DOI:10.1186/s12931-024-02664-x
24. Fan JJ, Gu JM, Xiao SY, et al. Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study. Front Med (Lausanne). 2024;11:1335758. DOI:10.3389/fmed.2024.1335758
25. Mendoza OM, Aldaheri S, Alyami S. Evaluation of Dyspnea Questionnaires in Patients with Idiopathic Pulmonary Fibrosis (IPF). European Respiratory Journal. 2018;52(Suppl. 62):PA2904. DOI:10.1183/13993003.congress-2018.PA2904
26. Öz M, Erol S, Küçükşahin O, et al. Clinical Functional, and Prognostic Evaluation of Idiopathic Pulmonary Fibrosis, Connective Tissue Disease-Associated Interstitial Lung Disease, Interstitial Pneumonia with Autoimmune Features: A Single-Center Prospective Study. Turk Thorac J. 2022;23:395-402. DOI:10.5152/TurkThoracJ.2022.22017
27. Sato R, Handa T, Matsumoto H, et al. Clinical significance of self-reported cough intensity and frequency in patients with interstitial lung disease: a cross-sectional study. BMC Pulm Med. 2019;19(1):247. DOI:10.1186/s12890-019-1012-6
28. Veit T, Barnikel M, Kneidinger N, et al. Clinical Impact of Physical Activity and Cough on Disease Progression in Fibrotic Interstitial Lung Disease. J Clin Med. 2023;12(11):3787. DOI:10.3390/jcm12113787
29. Cheng JZ, Wilcox PG, Glaspole I, et al. Cough is less common and less severe in systemic sclerosis-associated interstitial lung disease compared to other fibrotic interstitial lung diseases. Respirology. 2017;22(8):1592-7. DOI:10.1111/resp.13084
30. Yamamura K, Hara J, Watanabe S, et al. Patients with idiopathic pulmonary fibrosis and refractory cough have traction bronchiectasis and distorted airway architecture: a retrospective case review study. J Thorac Dis. 2024;16(3):2159-66. DOI:10.21037/jtd-23-1443
2. O'Brien EC, Hellkamp AS, Neely ML, et al. IPF-PRO Registry investigators. Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry. Chest. 2020;157(5):1188-98. DOI:10.1016/j.chest.2019.11.042
3. Fernández Pérez ER, Swigris JJ, Forssén AV, et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest. 2013;144(5):1644-51. DOI:10.1378/chest.12-2685
4. Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69. DOI:10.1164/rccm.202005-2032ST
5. Liao YW, Chen YM, Liu MC, et al. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024;29(1):69. DOI:10.1186/s40001-024-01644-7
6. Lee J, White E, Freiheit E, et al. Pulmonary Fibrosis Foundation. Cough-Specific Quality of Life Predicts Disease Progression Among Patients With Interstitial Lung Disease: Data From the Pulmonary Fibrosis Foundation Patient Registry. Chest. 2022;162(3):603-13. DOI:10.1016/j.chest.2022.03.025
7. Khor YH, Johannson KA, Marcoux V, et al. CARE-PF Investigators. Epidemiology and Prognostic Significance of Cough in Fibrotic Interstitial Lung Disease. Am J Respir Crit Care Med. 2024;210(8):1035-44. DOI:10.1164/rccm.202311-2101OC
8. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, et al. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis. PLoS One. 2016;11(3):e0151425. DOI:10.1371/journal.pone.0151425
9. Seixas E, Ferreira M, Serra P, et al. Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort. Afr J Thorac Crit Care Med. 2022;28(4). DOI:10.7196/AJTCCM.2022.v28i4.250
10. Adams TN, Batra K, Kypreos M, Glazer CS. Impact of radiographic honeycombing on transplant free survival and efficacy of immunosuppression in fibrotic hypersensitivity pneumonitis. BMC Pulm Med. 2023;23(1):224. DOI:10.1186/s12890-023-02523-3
11. Авдеев С.Н., Айсанов З.Р., Визель А.А., и др. Гиперчувствительный пневмонит: федеральные клинические рекомендации по диагностике и лечению. Пульмонология. 2025;35(1):16-41 [Avdeev SN, Aisanov ZR, Vizel AA, et al. Federal clinical guidelines on diagnosis and treatment of hypersensitivity pneumonitis. Pulmonology. 2025;35(1):16-41 (in Russian)]. DOI:10.18093/0869-0189-2025-35-1-16-41
12. Суворова О.А., Трушенко Н.В., Самсонова М.В., и др. Гиперчувствительный пневмонит: современное состояние проблемы. Медицина труда и промышленная экология. 2024;63(6):408-19 [Suvorova OA, Trushenko NV, Samsonova MV, et al. Hypersensitivity pneumonitis: the current state of the problem. Russian Journal of Occupational Health and Industrial Ecology. 2024;63(6):408-19 (in Russian)]. DOI:10.31089/1026-9428-2024-64-6-408-419
13. Raghu G, Remy-Jardin M, Myers JL, et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. DOI:10.1164/rccm.201807-1255ST
14. Fletcher CM, Elmes PC, Fairbairn AS, Wood CH. The significance of respiratory symptoms and the diagnosis of chronic bronchitis in a working population. Br Med J. 1959;2:256-66. DOI:10.1136/bmj.2.5147.257
15. Birring SS, Prudon B, Carr AJ, et al. Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax. 2003;58(4):339-43. DOI:10.1136/thorax.58.4.339
16. Charlson ME, Pompei P, Ales KL, MacKenzie CR. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis. 1987;40(5):373-83. DOI:10.1016/0021-9681(87)90171-8
17. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-91. DOI:10.7326/0003-4819-156-10-201205150-00004
18. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. DOI:10.1183/13993003.01499-2021
19. Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167(7):962-9. DOI:10.1164/rccm.2111053
20. Alberti ML, Malet Ruiz JM, Fernández ME, et al. Comparative survival analysis between idiopathic pulmonary fibrosis and chronic hypersensitivity pneumonitis. Pulmonology. 2020;26(1):3-9. DOI:10.1016/j.pulmoe.2019.08.007
21. Maher TM, Brown KK, Kreuter M, et al. INBUILD trial investigators. Effects of nintedanib by inclusion criteria for progression of interstitial lung disease. Eur Respir J. 2022;59(2):2004587. DOI:10.1183/13993003.04587-2020
22. Juliá-Serdá G, Navarro-Esteva J, Doreste-Salgado L, et al. Survival and Lung Function Changes in Hypersensitivity Pneumonitis According to Radiological Phenotypes Compared With Idiopathic Pulmonary Fibrosis. Cureus. 2024;16(3):e57307. DOI:10.7759/cureus.57307
23. Petnak T, Cheungpasitporn W, Thongprayoon C, et al. Phenotypic subtypes of fibrotic hypersensitivity pneumonitis identified by machine learning consensus clustering analysis. Respir Res. 2024;25(1):41. DOI:10.1186/s12931-024-02664-x
24. Fan JJ, Gu JM, Xiao SY, et al. Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study. Front Med (Lausanne). 2024;11:1335758. DOI:10.3389/fmed.2024.1335758
25. Mendoza OM, Aldaheri S, Alyami S. Evaluation of Dyspnea Questionnaires in Patients with Idiopathic Pulmonary Fibrosis (IPF). European Respiratory Journal. 2018;52(Suppl. 62):PA2904. DOI:10.1183/13993003.congress-2018.PA2904
26. Öz M, Erol S, Küçükşahin O, et al. Clinical Functional, and Prognostic Evaluation of Idiopathic Pulmonary Fibrosis, Connective Tissue Disease-Associated Interstitial Lung Disease, Interstitial Pneumonia with Autoimmune Features: A Single-Center Prospective Study. Turk Thorac J. 2022;23:395-402. DOI:10.5152/TurkThoracJ.2022.22017
27. Sato R, Handa T, Matsumoto H, et al. Clinical significance of self-reported cough intensity and frequency in patients with interstitial lung disease: a cross-sectional study. BMC Pulm Med. 2019;19(1):247. DOI:10.1186/s12890-019-1012-6
28. Veit T, Barnikel M, Kneidinger N, et al. Clinical Impact of Physical Activity and Cough on Disease Progression in Fibrotic Interstitial Lung Disease. J Clin Med. 2023;12(11):3787. DOI:10.3390/jcm12113787
29. Cheng JZ, Wilcox PG, Glaspole I, et al. Cough is less common and less severe in systemic sclerosis-associated interstitial lung disease compared to other fibrotic interstitial lung diseases. Respirology. 2017;22(8):1592-7. DOI:10.1111/resp.13084
30. Yamamura K, Hara J, Watanabe S, et al. Patients with idiopathic pulmonary fibrosis and refractory cough have traction bronchiectasis and distorted airway architecture: a retrospective case review study. J Thorac Dis. 2024;16(3):2159-66. DOI:10.21037/jtd-23-1443
________________________________________________
2. O'Brien EC, Hellkamp AS, Neely ML, et al. IPF-PRO Registry investigators. Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry. Chest. 2020;157(5):1188-98. DOI:10.1016/j.chest.2019.11.042
3. Fernández Pérez ER, Swigris JJ, Forssén AV, et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest. 2013;144(5):1644-51. DOI:10.1378/chest.12-2685
4. Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69. DOI:10.1164/rccm.202005-2032ST
5. Liao YW, Chen YM, Liu MC, et al. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024;29(1):69. DOI:10.1186/s40001-024-01644-7
6. Lee J, White E, Freiheit E, et al. Pulmonary Fibrosis Foundation. Cough-Specific Quality of Life Predicts Disease Progression Among Patients With Interstitial Lung Disease: Data From the Pulmonary Fibrosis Foundation Patient Registry. Chest. 2022;162(3):603-13. DOI:10.1016/j.chest.2022.03.025
7. Khor YH, Johannson KA, Marcoux V, et al. CARE-PF Investigators. Epidemiology and Prognostic Significance of Cough in Fibrotic Interstitial Lung Disease. Am J Respir Crit Care Med. 2024;210(8):1035-44. DOI:10.1164/rccm.202311-2101OC
8. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, et al. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis. PLoS One. 2016;11(3):e0151425. DOI:10.1371/journal.pone.0151425
9. Seixas E, Ferreira M, Serra P, et al. Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort. Afr J Thorac Crit Care Med. 2022;28(4). DOI:10.7196/AJTCCM.2022.v28i4.250
10. Adams TN, Batra K, Kypreos M, Glazer CS. Impact of radiographic honeycombing on transplant free survival and efficacy of immunosuppression in fibrotic hypersensitivity pneumonitis. BMC Pulm Med. 2023;23(1):224. DOI:10.1186/s12890-023-02523-3
11. Avdeev SN, Aisanov ZR, Vizel AA, et al. Federal clinical guidelines on diagnosis and treatment of hypersensitivity pneumonitis. Pulmonology. 2025;35(1):16-41 (in Russian). DOI:10.18093/0869-0189-2025-35-1-16-41
12. Suvorova OA, Trushenko NV, Samsonova MV, et al. Hypersensitivity pneumonitis: the current state of the problem. Russian Journal of Occupational Health and Industrial Ecology. 2024;63(6):408-19 (in Russian). DOI:10.31089/1026-9428-2024-64-6-408-419
13. Raghu G, Remy-Jardin M, Myers JL, et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. DOI:10.1164/rccm.201807-1255ST
14. Fletcher CM, Elmes PC, Fairbairn AS, Wood CH. The significance of respiratory symptoms and the diagnosis of chronic bronchitis in a working population. Br Med J. 1959;2:256-66. DOI:10.1136/bmj.2.5147.257
15. Birring SS, Prudon B, Carr AJ, et al. Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax. 2003;58(4):339-43. DOI:10.1136/thorax.58.4.339
16. Charlson ME, Pompei P, Ales KL, MacKenzie CR. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis. 1987;40(5):373-83. DOI:10.1016/0021-9681(87)90171-8
17. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-91. DOI:10.7326/0003-4819-156-10-201205150-00004
18. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. DOI:10.1183/13993003.01499-2021
19. Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167(7):962-9. DOI:10.1164/rccm.2111053
20. Alberti ML, Malet Ruiz JM, Fernández ME, et al. Comparative survival analysis between idiopathic pulmonary fibrosis and chronic hypersensitivity pneumonitis. Pulmonology. 2020;26(1):3-9. DOI:10.1016/j.pulmoe.2019.08.007
21. Maher TM, Brown KK, Kreuter M, et al. INBUILD trial investigators. Effects of nintedanib by inclusion criteria for progression of interstitial lung disease. Eur Respir J. 2022;59(2):2004587. DOI:10.1183/13993003.04587-2020
22. Juliá-Serdá G, Navarro-Esteva J, Doreste-Salgado L, et al. Survival and Lung Function Changes in Hypersensitivity Pneumonitis According to Radiological Phenotypes Compared With Idiopathic Pulmonary Fibrosis. Cureus. 2024;16(3):e57307. DOI:10.7759/cureus.57307
23. Petnak T, Cheungpasitporn W, Thongprayoon C, et al. Phenotypic subtypes of fibrotic hypersensitivity pneumonitis identified by machine learning consensus clustering analysis. Respir Res. 2024;25(1):41. DOI:10.1186/s12931-024-02664-x
24. Fan JJ, Gu JM, Xiao SY, et al. Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study. Front Med (Lausanne). 2024;11:1335758. DOI:10.3389/fmed.2024.1335758
25. Mendoza OM, Aldaheri S, Alyami S. Evaluation of Dyspnea Questionnaires in Patients with Idiopathic Pulmonary Fibrosis (IPF). European Respiratory Journal. 2018;52(Suppl. 62):PA2904. DOI:10.1183/13993003.congress-2018.PA2904
26. Öz M, Erol S, Küçükşahin O, et al. Clinical Functional, and Prognostic Evaluation of Idiopathic Pulmonary Fibrosis, Connective Tissue Disease-Associated Interstitial Lung Disease, Interstitial Pneumonia with Autoimmune Features: A Single-Center Prospective Study. Turk Thorac J. 2022;23:395-402. DOI:10.5152/TurkThoracJ.2022.22017
27. Sato R, Handa T, Matsumoto H, et al. Clinical significance of self-reported cough intensity and frequency in patients with interstitial lung disease: a cross-sectional study. BMC Pulm Med. 2019;19(1):247. DOI:10.1186/s12890-019-1012-6
28. Veit T, Barnikel M, Kneidinger N, et al. Clinical Impact of Physical Activity and Cough on Disease Progression in Fibrotic Interstitial Lung Disease. J Clin Med. 2023;12(11):3787. DOI:10.3390/jcm12113787
29. Cheng JZ, Wilcox PG, Glaspole I, et al. Cough is less common and less severe in systemic sclerosis-associated interstitial lung disease compared to other fibrotic interstitial lung diseases. Respirology. 2017;22(8):1592-7. DOI:10.1111/resp.13084
30. Yamamura K, Hara J, Watanabe S, et al. Patients with idiopathic pulmonary fibrosis and refractory cough have traction bronchiectasis and distorted airway architecture: a retrospective case review study. J Thorac Dis. 2024;16(3):2159-66. DOI:10.21037/jtd-23-1443
Авторы
О.А. Суворова*1, Н.В. Трушенко1,2, Б.Б. Лавгинова1, Ю.А. Левина1, З.М. Мержоева1, С.Н. Авдеев1,2
1ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2ФГБУ «Научно-исследовательский институт пульмонологии» ФМБА России, Москва, Россия
*olga.a.suvorova@mail.ru
1Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
2Research Institute of Pulmonology, Moscow, Russia
*olga.a.suvorova@mail.ru
1ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2ФГБУ «Научно-исследовательский институт пульмонологии» ФМБА России, Москва, Россия
*olga.a.suvorova@mail.ru
________________________________________________
1Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
2Research Institute of Pulmonology, Moscow, Russia
*olga.a.suvorova@mail.ru
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