Легочная гипертензия при саркоидозе
Аннотация
Легочная гипертензия (ЛГ) является относительно частым осложнением саркоидоза. По разным данным, распространенность ЛГ при саркоидозе составляет 6–74 %. ЛГ, ассоциированная с саркоидозом (ЛГАС), как правило, связана с довольно плохим прогнозом. Несмотря на то, что ЛГАС чаще всего встречается у пациентов с тяжелыми стадиями саркоидоза, иногда она развивается при отсутствии поражения легочной паренхимы. Патофизиология развития ЛГАС довольно сложна и включает несколько различных механизмов, таких как легочный фиброз, гипоксическая вазоконстрикция, гранулематозная облитерация и / или ангиит легочных сосудов, внешняя компрессия легочных сосудов, легочная веноокклюзионная болезнь и поражение левых отделов сердца. Ведение пациентов с ЛГАС основано на устранении гипоксемии, лечении активного саркоидоза и сопутствующих заболеваний. Место системных глюкокортикостероидов в терапии ЛГАС остается противоречивым. Специфическая терапия легочной артериальной гипертензии (ЛАГ) пока не рекомендована для всех пациентов с ЛГАС, хотя в ряде небольших исследований у некоторых больных продемонстрирован положительный эффект применения простаноидов, антагонистов рецепторов эндотелина и ингибиторов фосфодиэстеразы5. Для подтверждения роли ЛАГспецифичной терапии у определенных групп больных ЛГАС необходимо проведение новых, хорошо спланированных рандомизированных плацебоконтролируемых исследований.
Об авторе
С. Н. Авдеев
Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства России»
Россия
Россия
105077, Россия, Москва, ул. 11&я Парковая, 32, корп. 4
д. м. н., профессор, членкорр. Российской академии наук, руководитель клинического отдела Федерального государственного бюджетного учреждения «Научно-исследовательский институт пульмонологии Федерального медикобиологического агентства России»; тел.: (495) 4655264
Список литературы
1. Simonneau G., Gatzoulis M.A., Adatia I. et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62: D34–41.
2. Cordova F.C., D'Alonzo G. Sarcoidosisassociated pulmonary hypertension. Curr. Opin. Pulm. Med. 2013; 19: 531–537.
3. Battesti J.P., Georges R., Basset F., Saumon G. Chronic corpulmonale in pulmonary sarcoidosis. Thorax. 1978; 33: 76–84.
4. Głuskowski J., Hawryłkiewicz I., Zych D. et al. Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis. Respiration. 1984; 46: 26–32.
5. Handa T., Nagai S., Miki S. et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006; 129: 1246–1252.
6. Milman N., Burton C.M., Iversen M. et al. Pulmonary hypertension in endstage pulmonary sarcoidosis: therapeutic effect of sildenafil? J. Heart Lung Transplant. 2008; 27: 329–334.
7. Mitchell D.N., Scadding J.G. Sarcoidosis. Am. Rev. Respir. Dis. 1974; 110 (6): 774–802.
8. Rizzato G., Pezzano A., Sala G. et al. Right heart impairment in sarcoidosis: haemodynamic and echocardiographic study. Eur. J. Respir. Dis. 1983; 64: 121–128.
9. Shorr A.F., Davies D.B., Nathan S.D. Outcomes for patients with sarcoidosis awaiting lung transplantation. Chest. 2002; 122: 233–238.
10. Sulica R., Teirstein A.S., Kakarla S. et al. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosisrelated pulmonary hypertension. Chest. 2005; 128: 1483–1489.
11. Nunes H., Humbert M., Capron F. et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemody namics and prognosis. Thorax. 2006; 61: 68–74
12. Shah L. Lung transplantation in sarcoidosis. Semin. Respir. Crit. Care Med. 2007; 28: 134–140.
13. Bourbonnais J.M., Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur. Respir. J. 2008; 32: 296–302.
14. Maimon N., Salz L., Shershevsky Y. et al. D. Sarcoidosis associated pulmonary hypertension in patients with nearnormal lung function. Int. J. Tuberc. Lung Dis. 2013; 17: 406–411.
15. Arcasoy S.M., Christie J.D., Pochettino A. et al. Characteristics and outcomes of patients with sarcoidosis listed for lung transplantation. Chest. 2001; 120: 873–880.
16. Shorr A.F., Helman D.L., Davies D.B., Nathan S.D. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur. Respir. J. 2005; 25: 783–788.
17. Galiè N., Hoeper M.M., Humbert M. et al.Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur. Heart J. 2009; 30: 2493–2537.
18. Lahm T., Chakinala M.M. World Health Organization group 5 pulmonary hypertension. Clin. Chest Med. 2013; 34: 753–778.
19. Preston I.R., Klinger J.R., Landzberg M.J. et al. Vasores ponsiveness of sarcoidosisassociated pulmonary hypertension. Chest. 2001; 120: 866–872.
20. Takemura T., Matsui Y., Oritsu M. et al. Pulmonary vascular involvement in sarcoidosis: granulomatous angiitis and microangiopathy in transbronchial lung biopsies. Virchows. Arch. Pathol. Anat. Histopathol. 1991; 418: 361–368.
21. Takemura T., Matsui Y., Saiki S., Mikami R. Pulmonary vascular involvement in sarcoidosis: a report of 40 autopsycases. Hum. Pathol. 1992; 23: 1216–1223.
22. Rosen Y., Moon S., Huang C.T. et al. Granulomatous pulmonary angiitis in sarcoidosis. Arch. Pathol. Lab. Med. 1977; 101: 170–174.
23. Carrington C.B. Structure and function in sarcoidosis. Ann. NY Acad. Sci. 1976; 278: 265–283.
24. Rosen Y. Pathology of sarcoidosis. Semin. Respir. Crit. Care Med. 2007; 28 (1): 36–52.
25. Shorr A.F., Davies D.B., Nathan S.D. Predicting mortality in patients with sarcoidosis awaiting lung transplantation. Chest. 2003; 124: 922–928.
26. Damuth T.E., Bower J.S., Cho K., Dantzker D.R. Major pulmonary artery stenosis causing pulmonary hypertension in sarcoidosis. Chest. 1980; 78: 888–891.
27. Gaston B., Drazen J.M., Loscalzo J., Stamler J.S. The biology of nitrogen oxides in the airways. Am. J. Respir. Crit. Care Med. 1994; 149: 538–551.
28. Giaid A., Saleh D. Reduced expression of endothelial nitricoxide synthase in the lungs of patients with pulmonary hypertension. N. Engl. J. Med. 1995; 333: 214–221.
29. Milman N., Svendsen C.B., Iversen M. et al. Sarcoidosis associated pulmonary hypertension: acute vasoresponsive ness to inhaled nitric oxide and the relation to longterm effect of sildenafil. Clin. Respir. J. 2009; 3: 207–213.
30. Galiè N., Manes A., Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc. Res. 2004; 61: 227–237.
31. Giaid A., Yanagisawa M., Langleben D. et al. Expression of endothelin1 in the lungs of patients with pulmonary hypertension. N. Engl. J. Med. 1993; 328: 1732–1739.
32. Sofia M., Mormile M., Faraone S. et al. Endothelin1 excretion in urine in active pulmonary sarcoidosis and in other interstitial lung diseases. Sarcoidosis. 1995; 12: 118–123.
33. Reichenberger F., Schauer J., Kellner K. et al. Different expression of endothelin in the bronchoalveolar lavage in patients with pulmonary diseases. Lung. 2001; 179: 163–174.
34. Terashita K., Kato S., Sata M. et al. Increased endothelin1 levels of BAL fluid in patients with pulmonary sarcoidosis. Respirology. 2006; 11: 145–151.
35. Letizia C., Danese A., Reale M.G. et al. Plasma levels of endothelin1 increase in patients with sarcoidosis and fall after disease remission. Panminerva. Med. 2001; 43: 257–261.
36. Barnett C.F., Bonura E.J., Nathan S.D. et al. Treatment of sarcoidosisassociated pulmonary hypertension. A twocen ter experience. Chest. 2009; 135: 1455–1461.
37. Baughman R.P., Engel P.J., Meyer C.A. et al. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2006; 23: 108–116.
38. Foley R.J., Metersky M.L. Successful treatment of sarcoidosisassociated pulmonary hypertension with bosentan. Respiration. 2008; 75: 211–214.
39. Hoffstein V., Ranganathan N., Mullen J.B. Sarcoidosis simulating pulmonary venoocclusive disease. Am. Rev. Respir. Dis. 1986; 134: 809–811.
40. Jones R.M., Dawson A., Jenkins G.H. et al. Sarcoidosis related pulmonary venoocclusive disease presenting with recurrent haemoptysis. Eur. Respir. J. 2009; 34: 517–520.
41. Portier F., LereboursPigeonniere G., Thiberville L. et al. Sarcoidosis simulating a pulmonary venoocclusive disease. Rev. Mal. Respir. 1991; 8: 101–102.
42. Wagenvoort C.A., Wagenvoort N. The pathology of pulmonary venoocclusive disease. Virchows. Arch. Pathol. Anat. Histol. 1974; 364: 69–79.
43. Montani D., O'Callaghan D.S., Savale L. et al. Pulmonary venoocclusive disease: recent progress and current challenges. Respir. Med. 2010; 104 (Suppl. 1): S23–S32.
44. Roberts W.C., McAllister H.A. Jr, Ferrans V.J. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group I) and review of 78 previously described necropsy patients (group II). Am. J. Med. 1977; 63: 86–108.
45. Mehta D., Lubitz S.A., Frankel Z. et al. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest. 2008; 133: 1426–1435.
46. Perry A., Vuitch F. Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations. Arch. Pathol. Lab. Med. 1995; 119: 167–172.
47. Iwai K., Sekiguti M., Hosoda Y. et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis. 1994; 11: 26–31.
48. Saggar R., Saggar R., Aboulhosn J. et al. Diagnosis and hemodynamic assessment of pulmonary arterial hypertension. Semin. Respir. Crit. Care Med. 2009; 30: 399–410.
49. Hoeper M.M., Lee S.H., Voswinckel R. et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J. Am. Coll. Cardiol. 2006; 48: 2546–2552.
50. Arcasoy S.M., Christie J.D., Ferrari V.A. et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am. J. Respir. Crit. Care Med. 2003; 167: 735–740.
51. Nathan S.D., Shlobin O.A., Barnett S.D. et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir. Med. 2008; 102: 1305–1310.
52. Zisman D.A., Ross D.J., Belperio J.A. et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir. Med. 2007; 101: 2153–2159.
53. Rich J.D., Shah S.J., Swamy R.S. et al. Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest. 2011; 139: 988–993.
54. Devaraj A., Wells A.U., Meister M.G. et al. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology. 2008; 249: 1042–1049.
55. Shino M.Y., Lynch J.P., Fishbein M.C. Sarcoidosisassociated pulmonary hypertension and lung transplantation for sarcoidosis. Semin. Respir. Crit. Care Med. 2014; 35: 362–371.
56. Emirgil C., Sobol B.J., Herbert W.H., Trout K. The lesser circulation in pulmonary fibrosis secondary to sarcoidosis and its relationship to respiratory function. Chest. 1971; 60: 371–378.
57. Handa T., Nagai S., Ueda S. et al. Significance of plasma NTproBNP levels as a biomarker in the assessment of cardiac involvement and pulmonary hypertension in patients with sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2010; 27: 27–35.
58. Iannuzzi M.C., Rybicki B.A., Teirstein A.S. Sarcoidosis. N. Engl. J. Med. 2007; 357: 2153–2165.
59. Lynch J.P. III, Kazerooni E.A., Gay S.E. Pulmonary sarcoidosis. Clin. Chest Med. 1997; 18: 755–785.
60. Macfarlane J.T. Prognosis in sarcoidosis. Br. Med. J. (Clin. Res. Ed.) 1984; 288: 1557–1558.
61. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am. J. Respir. Crit. Care Med. 1999; 160: 736–755.
62. Baughman R.P. Pulmonary sarcoidosis. Clin. Chest Med. 2004; 25: 521–530.
63. Patterson K.C., Strek M.E. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann. Am. Thorac. Soc. 2013; 10: 362–370.
64. Lynch J.P. III, Ma Y.L., Koss M.N., White E.S. Pulmonary sarcoidosis. Semin. Respir. Crit. Care Med. 2007; 28: 53–74.
65. Gluskowski J., Hawrylkiewicz I., Zych D., Zieliński J. Effects of corticosteroid treatment on pulmonary haemodynamics in patients with sarcoidosis. Eur. Respir. J. 1990; 3: 403–407.
66. Pitsiou G., Papakosta D., Bouros D. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Respiration. 2011; 82: 294–304.
67. Patel N.M., Lederer D.J., Borczuk A.C., Kawut S.M. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. 2007; 132: 998–1006.
68. Fisher K.A., Serlin D.M., Wilson K.C. et al. Sarcoidosis associated pulmonary hypertension: outcome with long term epoprostenol treatment. Chest. 2006; 130: 1481–1488.
69. Olschewski H., Ghofrani H.A., Walmrath D. et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am. J. Respir. Crit. Care Med. 1999; 160: 600–607.
70. Olschewski H. Inhaled iloprost for the treatment of pulmonary hypertension. Eur. Respir. Rev. 2009; 18: 29–34.
71. Baughman R.P., Judson M.A., Lower E.E. et al. Inhaled iloprost for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc. Diffuse Lung Dis. 2009; 26: 110–120.
72. Baughman R.P. Pulmonary hypertension associated with sarcoidosis. Arthritis Res. Ther. 2007; 9 (Suppl. 2): S8.
73. Sharma S., Kashour T., Philipp R. Secondary pulmonary arterial hypertension: treated with endothelin receptor blockade. Tex. Heart Inst. J. 2005; 32: 405–410.
74. Baughman R.P., Culver D.A., Cordova F.C. et al. Bosentan for sarcoidosis associated pulmonary hypertension: A doubleblind placebo controlled randomized trial. Chest. 2014; 145: 810–817.
75. Dobarro D., Schreiber B.E., Handler C. et al. Clinical characteristics, haemodynamics and treatment of pulmonary hypertension in sarcoidosis in a single centre, and metaanalysis of the published data. Am. J. Cardiol. 2013; 111: 278–285.
76. Barnett C.F., Bonura E.J., Nathan S.D. et al. Treatment of sarcoidosisassociated pulmonary hypertension. A twocenter experience. Chest. 2009; 135: 1455–1461.
77. Baughman R.P., Engel P.J., Meyer C.A. et al. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2006; 23: 108–116.
78. Foley R.J., Metersky M.L. Successful treatment of sarcoidosisassociated pulmonary hypertension with bosentan. Respiration. 2008; 75: 211–214.
79. Judson M.A., Highland K.B., Kwon S. et al. Ambrisentan for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc. Diffuse Lung Dis. 2011; 28: 139–145.
Для цитирования:
Авдеев С.Н. Легочная гипертензия при саркоидозе. Пульмонология. 2016;26(6):725-735. https://doi.org/10.18093/0869-0189-2016-26-6-725-735
For citation:
Avdeev S.N. Pulmonary hypertension in sarcoidosis. PULMONOLOGIYA. 2016;26(6):725-735. (In Russ.) https://doi.org/10.18093/0869-0189-2016-26-6-725-735
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