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Диагностика лимфангиолейомиоматоза: роль хирургической биопсии легкого. Клинические случаи
Диагностика лимфангиолейомиоматоза: роль хирургической биопсии легкого. Клинические случаи
Макарова М.А., Черняк А.В., Карнозова Т.Н., Бродская О.Н., Красовский С.А. Диагностика лимфангиолейомиоматоза: роль хирургической биопсии легкого. Клинические случаи. Consilium Medicum. 2021; 23 (3): 285–291. DOI: 10.26442/20751753.2021.3.200753
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Аннотация
В статье представлено 2 клинических наблюдения постановки диагноза лимфангиолейомиоматоза без хирургической биопсии легкого.
Ключевые слова: лимфангиолейомиоматоз, эндотелиальный фактор роста сосудов D, ангиомиолипома, лимфангиолейомиома, спирометрия, диффузионная способность легких, FEV1, DLco
Keywords: lymphangioleiomyomatosis, vascular endothelial growth factor D, angiomyolipoma, lymphangioleiomyoma, spirometry, diffusion capacity, FEV1, DLco
Ключевые слова: лимфангиолейомиоматоз, эндотелиальный фактор роста сосудов D, ангиомиолипома, лимфангиолейомиома, спирометрия, диффузионная способность легких, FEV1, DLco
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Keywords: lymphangioleiomyomatosis, vascular endothelial growth factor D, angiomyolipoma, lymphangioleiomyoma, spirometry, diffusion capacity, FEV1, DLco
Полный текст
Список литературы
1. Henske EP, McCormack FX. Lymphangioleiomyomatosis: a wolf in sheep’s clothing. J Clin Invest. 2012; 122: 3807–16. DOI: 10.1172/JCI58709
2. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 Health Organization classification of lung tumors: impactof genetic, clinical and radiologic advances since the 2004 classi fication. J Thorac Oncol. 2015; 10: 1243–60. DOI: 10.1097/JTO.0000000000000630
3. Sengupta S, Peterson TR, Sabatini DM. Regulation of the mTOR complex 1 pathway by nutrients, growth factors, and stress. Mol Cell. 2010; 40: 310–22. DOI: 10.1016/j.molcel.2010.09.026
4. Kumasaka T, Seyama K, Mitani K, et al. Lymphangiogenesismediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol. 2005; 29: 1356–66. DOI: 10.1097/01.pas.0000172192.25295.45
5. Chang WY, Cane JL, Blakey JD, et al. Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis. Respir Res. 2012; 13: 34. DOI: 10.1186/1465-9921-13-34
6. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010; 35: 14–26. DOI: 10.1183/09031936.00076209
7. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. ATS/JRS Committee on Lymphangioleiomyomatosis. Am J Respir Crit Care Med. 2016; 194 (6): 748–61. DOI: 10.1164/rccm.201607-1384ST
8. Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. ATS Assembly on Clinical Problems. J Respir Crit Care Med. 2017; 196 (10): 1337–48.
DOI: 10.1164/rccm.201709-1965ST
9. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium. MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364 (17): 1595–606. DOI: 10.1056/NEJMoa1100391
10. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005; 26: 948–68. DOI: 10.1183/09031936.05.00035205
11. Неклюдова Г.В., Черняк А.В. Диагностические возможности исследования диффузионной способности легких. Мед. алфавит. 2020; 25: 22–5 [Nekliudova GV, Cherniak AV. Diagnosticheskie vozmozhnosti issledovaniia diffuzionnoi sposobnosti legkikh. Med. alfavit. 2020; 25: 22–5 (in Russian)]. DOI: 10.33667/2078–5631–2020–25–22–25
12. McLean A, Warren PM, Gillooly M, et al. Microscopic and macroscopic measurements of emphysema: relation to carbon monoxide gas transfer. Thorax. 1992; 47: 144–9. DOI: 10.1136/thx.47.3.144
13. Черняк А.В., Макарова М.А., Авдеев С.Н. Функция внешнего дыхания у больных лимфангиолейомиоматозом. Бюл. физиологии и патологии дыхания. 2021; 79: 21–31 [Cherniak AV, Makarova MA, Avdeev SN. Lung function in patients with lymphangioleiomyomatosis. Bulletin Physiology and Pathology of Respiration. 2021; 79: 21–31 (in Russian)]. DOI: 10.36604/1998-5029-2021-79-21-31
14. Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax. 2005; 60: 875. DOI: 10.1136/thx.2004.035154
15. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006; 173: 105. DOI: 10.1164/rccm.200409-1298OC
16. Тюрин И.Е. Визуализация хронической обструктивной болезни легких. Практ. пульмонология. 2014; 2: 40–6 [Tiurin IE. Vizualizatsiia khronicheskoi obstruktivnoi bolezni legkikh. Prakt. pul'monologiia. 2014; 2: 40–6 (in Russian)].
17. Hartman TE, Tazelaar HD, Swensen SJ, Muller NL. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997; 17 (2): 377–90. DOI: 10.1148/radiographics.17.2.9084079
18. Ferrans VJ, Yu ZX, Nelson WK, et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. J Nippon Med Sch. 2000; 67 (5): 311–29. DOI: 10.1272/jnms.67.311
2. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 Health Organization classification of lung tumors: impactof genetic, clinical and radiologic advances since the 2004 classi fication. J Thorac Oncol. 2015; 10: 1243–60. DOI: 10.1097/JTO.0000000000000630
3. Sengupta S, Peterson TR, Sabatini DM. Regulation of the mTOR complex 1 pathway by nutrients, growth factors, and stress. Mol Cell. 2010; 40: 310–22. DOI: 10.1016/j.molcel.2010.09.026
4. Kumasaka T, Seyama K, Mitani K, et al. Lymphangiogenesismediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol. 2005; 29: 1356–66. DOI: 10.1097/01.pas.0000172192.25295.45
5. Chang WY, Cane JL, Blakey JD, et al. Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis. Respir Res. 2012; 13: 34. DOI: 10.1186/1465-9921-13-34
6. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010; 35: 14–26. DOI: 10.1183/09031936.00076209
7. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. ATS/JRS Committee on Lymphangioleiomyomatosis. Am J Respir Crit Care Med. 2016; 194 (6): 748–61. DOI: 10.1164/rccm.201607-1384ST
8. Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. ATS Assembly on Clinical Problems. J Respir Crit Care Med. 2017; 196 (10): 1337–48.
DOI: 10.1164/rccm.201709-1965ST
9. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium. MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364 (17): 1595–606. DOI: 10.1056/NEJMoa1100391
10. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005; 26: 948–68. DOI: 10.1183/09031936.05.00035205
11. Неклюдова Г.В., Черняк А.В. Диагностические возможности исследования диффузионной способности легких. Мед. алфавит. 2020; 25: 22–5 [Nekliudova GV, Cherniak AV. Diagnosticheskie vozmozhnosti issledovaniia diffuzionnoi sposobnosti legkikh. Med. alfavit. 2020; 25: 22–5 (in Russian)]. DOI: 10.33667/2078–5631–2020–25–22–25
12. McLean A, Warren PM, Gillooly M, et al. Microscopic and macroscopic measurements of emphysema: relation to carbon monoxide gas transfer. Thorax. 1992; 47: 144–9. DOI: 10.1136/thx.47.3.144
13. Cherniak AV, Makarova MA, Avdeev SN. Lung function in patients with lymphangioleiomyomatosis. Bulletin Physiology and Pathology of Respiration. 2021; 79: 21–31 (in Russian) DOI: 10.36604/1998-5029-2021-79-21-31
14. Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax. 2005; 60: 875. DOI: 10.1136/thx.2004.035154
15. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006; 173: 105. DOI: 10.1164/rccm.200409-1298OC
16. Tiurin IE. Vizualizatsiia khronicheskoi obstruktivnoi bolezni legkikh. Prakt. pul'monologiia. 2014; 2: 40–6 (in Russian)
17. Hartman TE, Tazelaar HD, Swensen SJ, Muller NL. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997; 17 (2): 377–90. DOI: 10.1148/radiographics.17.2.9084079
18. Ferrans VJ, Yu ZX, Nelson WK, et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. J Nippon Med Sch. 2000; 67 (5): 311–29. DOI: 10.1272/jnms.67.311
2. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 Health Organization classification of lung tumors: impactof genetic, clinical and radiologic advances since the 2004 classi fication. J Thorac Oncol. 2015; 10: 1243–60. DOI: 10.1097/JTO.0000000000000630
3. Sengupta S, Peterson TR, Sabatini DM. Regulation of the mTOR complex 1 pathway by nutrients, growth factors, and stress. Mol Cell. 2010; 40: 310–22. DOI: 10.1016/j.molcel.2010.09.026
4. Kumasaka T, Seyama K, Mitani K, et al. Lymphangiogenesismediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol. 2005; 29: 1356–66. DOI: 10.1097/01.pas.0000172192.25295.45
5. Chang WY, Cane JL, Blakey JD, et al. Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis. Respir Res. 2012; 13: 34. DOI: 10.1186/1465-9921-13-34
6. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010; 35: 14–26. DOI: 10.1183/09031936.00076209
7. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. ATS/JRS Committee on Lymphangioleiomyomatosis. Am J Respir Crit Care Med. 2016; 194 (6): 748–61. DOI: 10.1164/rccm.201607-1384ST
8. Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. ATS Assembly on Clinical Problems. J Respir Crit Care Med. 2017; 196 (10): 1337–48.
DOI: 10.1164/rccm.201709-1965ST
9. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium. MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364 (17): 1595–606. DOI: 10.1056/NEJMoa1100391
10. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005; 26: 948–68. DOI: 10.1183/09031936.05.00035205
11. Неклюдова Г.В., Черняк А.В. Диагностические возможности исследования диффузионной способности легких. Мед. алфавит. 2020; 25: 22–5 [Nekliudova GV, Cherniak AV. Diagnosticheskie vozmozhnosti issledovaniia diffuzionnoi sposobnosti legkikh. Med. alfavit. 2020; 25: 22–5 (in Russian)]. DOI: 10.33667/2078–5631–2020–25–22–25
12. McLean A, Warren PM, Gillooly M, et al. Microscopic and macroscopic measurements of emphysema: relation to carbon monoxide gas transfer. Thorax. 1992; 47: 144–9. DOI: 10.1136/thx.47.3.144
13. Черняк А.В., Макарова М.А., Авдеев С.Н. Функция внешнего дыхания у больных лимфангиолейомиоматозом. Бюл. физиологии и патологии дыхания. 2021; 79: 21–31 [Cherniak AV, Makarova MA, Avdeev SN. Lung function in patients with lymphangioleiomyomatosis. Bulletin Physiology and Pathology of Respiration. 2021; 79: 21–31 (in Russian)]. DOI: 10.36604/1998-5029-2021-79-21-31
14. Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax. 2005; 60: 875. DOI: 10.1136/thx.2004.035154
15. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006; 173: 105. DOI: 10.1164/rccm.200409-1298OC
16. Тюрин И.Е. Визуализация хронической обструктивной болезни легких. Практ. пульмонология. 2014; 2: 40–6 [Tiurin IE. Vizualizatsiia khronicheskoi obstruktivnoi bolezni legkikh. Prakt. pul'monologiia. 2014; 2: 40–6 (in Russian)].
17. Hartman TE, Tazelaar HD, Swensen SJ, Muller NL. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997; 17 (2): 377–90. DOI: 10.1148/radiographics.17.2.9084079
18. Ferrans VJ, Yu ZX, Nelson WK, et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. J Nippon Med Sch. 2000; 67 (5): 311–29. DOI: 10.1272/jnms.67.311
________________________________________________
2. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 Health Organization classification of lung tumors: impactof genetic, clinical and radiologic advances since the 2004 classi fication. J Thorac Oncol. 2015; 10: 1243–60. DOI: 10.1097/JTO.0000000000000630
3. Sengupta S, Peterson TR, Sabatini DM. Regulation of the mTOR complex 1 pathway by nutrients, growth factors, and stress. Mol Cell. 2010; 40: 310–22. DOI: 10.1016/j.molcel.2010.09.026
4. Kumasaka T, Seyama K, Mitani K, et al. Lymphangiogenesismediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol. 2005; 29: 1356–66. DOI: 10.1097/01.pas.0000172192.25295.45
5. Chang WY, Cane JL, Blakey JD, et al. Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis. Respir Res. 2012; 13: 34. DOI: 10.1186/1465-9921-13-34
6. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010; 35: 14–26. DOI: 10.1183/09031936.00076209
7. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. ATS/JRS Committee on Lymphangioleiomyomatosis. Am J Respir Crit Care Med. 2016; 194 (6): 748–61. DOI: 10.1164/rccm.201607-1384ST
8. Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. ATS Assembly on Clinical Problems. J Respir Crit Care Med. 2017; 196 (10): 1337–48.
DOI: 10.1164/rccm.201709-1965ST
9. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium. MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364 (17): 1595–606. DOI: 10.1056/NEJMoa1100391
10. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005; 26: 948–68. DOI: 10.1183/09031936.05.00035205
11. Неклюдова Г.В., Черняк А.В. Диагностические возможности исследования диффузионной способности легких. Мед. алфавит. 2020; 25: 22–5 [Nekliudova GV, Cherniak AV. Diagnosticheskie vozmozhnosti issledovaniia diffuzionnoi sposobnosti legkikh. Med. alfavit. 2020; 25: 22–5 (in Russian)]. DOI: 10.33667/2078–5631–2020–25–22–25
12. McLean A, Warren PM, Gillooly M, et al. Microscopic and macroscopic measurements of emphysema: relation to carbon monoxide gas transfer. Thorax. 1992; 47: 144–9. DOI: 10.1136/thx.47.3.144
13. Cherniak AV, Makarova MA, Avdeev SN. Lung function in patients with lymphangioleiomyomatosis. Bulletin Physiology and Pathology of Respiration. 2021; 79: 21–31 (in Russian) DOI: 10.36604/1998-5029-2021-79-21-31
14. Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax. 2005; 60: 875. DOI: 10.1136/thx.2004.035154
15. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006; 173: 105. DOI: 10.1164/rccm.200409-1298OC
16. Tiurin IE. Vizualizatsiia khronicheskoi obstruktivnoi bolezni legkikh. Prakt. pul'monologiia. 2014; 2: 40–6 (in Russian)
17. Hartman TE, Tazelaar HD, Swensen SJ, Muller NL. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997; 17 (2): 377–90. DOI: 10.1148/radiographics.17.2.9084079
18. Ferrans VJ, Yu ZX, Nelson WK, et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. J Nippon Med Sch. 2000; 67 (5): 311–29. DOI: 10.1272/jnms.67.311
Авторы
М.А. Макарова*1,2, А.В. Черняк2,3, Т.Н. Карнозова4, О.Н. Бродская1, С.А. Красовский2,5
1 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, Москва, Россия;
2 ФГБУ «Научно-исследовательский институт пульмонологии» ФМБА России, Москва, Россия;
3 ГБУЗ «Городская клиническая больница им. Д.Д. Плетнева» Департамента здравоохранения г. Москвы, Москва, Россия;
4 ФГБУ «Федеральный клинический центр высоких медицинских технологий» ФМБА России, Химки, Россия;
5 ФГБНУ «Медико-генетический научный центр им. акад. Н.П. Бочкова», Москва, Россия
*mma123@list.ru
1 Pirogov Russian National Research Medical University, Moscow, Russia;
2 Research Institute of Pulmonology, Moscow, Russia;
3 Pletnev City Clinical Hospital, Moscow, Russia;
4 Federal Clinical Center for High Medical Technologies, Khimki, Russia;
5 Bochkov Medical Genetic Research Center, Moscow, Russia
*mma123@list.ru
1 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, Москва, Россия;
2 ФГБУ «Научно-исследовательский институт пульмонологии» ФМБА России, Москва, Россия;
3 ГБУЗ «Городская клиническая больница им. Д.Д. Плетнева» Департамента здравоохранения г. Москвы, Москва, Россия;
4 ФГБУ «Федеральный клинический центр высоких медицинских технологий» ФМБА России, Химки, Россия;
5 ФГБНУ «Медико-генетический научный центр им. акад. Н.П. Бочкова», Москва, Россия
*mma123@list.ru
________________________________________________
1 Pirogov Russian National Research Medical University, Moscow, Russia;
2 Research Institute of Pulmonology, Moscow, Russia;
3 Pletnev City Clinical Hospital, Moscow, Russia;
4 Federal Clinical Center for High Medical Technologies, Khimki, Russia;
5 Bochkov Medical Genetic Research Center, Moscow, Russia
*mma123@list.ru
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