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Легочная артериальная гипертензия, ассоциированная с системной склеродермией, и идиопатическая легочная гипертензия: сравнительный анализ клинико-демографических особенностей и выживаемости по данным Российского национального регистра
Легочная артериальная гипертензия, ассоциированная с системной склеродермией, и идиопатическая легочная гипертензия: сравнительный анализ клинико-демографических особенностей и выживаемости по данным Российского национального регистра
Юдкина Н.Н., Валеева Э.Г., Таран И.Н. и др. Легочная артериальная гипертензия, ассоциированная с системной склеродермией, и идиопатическая легочная гипертензия: сравнительный анализ клинико-демографических особенностей и выживаемости по данным Российского национального регистра. Системные гипертензии. 2016; 13 (2): 65–72.
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Аннотация
Легочная артериальная гипертензия, ассоциированная с системной склеродермией (ЛАГ-ССД), и идиопатическая легочная гипертензия (ИЛГ) относятся к группе I по классификации ЛГ, однако имеются данные о существенных различиях в их выживаемости на фоне современной терапии.
Цель: провести сравнительную характеристику пациентов с ЛАГ-ССД и ИЛГ по данным Российского национального регистра.
Материалы и методы: 52 больных ИЛГ и 50 – ЛАГ-ССД. Пациенты не различались по функциональному классу (ФК). Средние значения ФК в группах составили 2,7±0,8 и 2,6±0,7 соответственно. Диагноз устанавливался на основании катетеризации правых отделов сердца и легочной артерии.
Результаты. Пациенты с ЛАГ-ССД на момент постановки диагноза были в среднем на 15 лет старше, чем с ИЛГ (р<0,0001). Дистанция 6-минутного теста ходьбы в группе ИЛГ была несколько больше. Индекс Борга был выше в группе ЛАГ-ССД (4,2±2,1 vs 3,3±1,5); р=0,049. Степень недостаточности кровообращения (НК) и дыхательной недостаточности не различались. Самым частым первым симптомом ЛАГ была одышка (94% – при ЛАГ-ССД и 73% – при ИЛГ). Обмороки выявлялись только при ИЛГ (13%). Диагноз ЛАГ в обеих группах устанавливался поздно [при ЛАГ-ССД – через 33 (13; 56,5), при ИЛГ – через 30,5 (13,3; 76,3) мес]. Признаки НК на момент установления диагноза выявлялись с одинаковой частотой как при ЛАГ-ССД, так и ИЛГ (76 и 65% соответственно). Проявления развернутой стадии заболевания, включая признаки НК, выявлялись с одинаковой частотой. Лишь при эхокардиографии у пациентов c ЛАГ-ССД чаще обнаруживался перикардиальный выпот (56 и 35%); р<0,05. Больные с ССД имели достоверно более низкую массу тела (14 и 2%); р<0,05. При оценке центральной гемодинамики обнаружены достоверные различия: давление в правом желудочке (ДПЖ) систолическое (73,4±19,1 мм рт. ст.) и среднее давление в легочной артерии – ДЛАср (49,1±11,5 мм рт. ст.) при ЛАГ-ССД было достоверно ниже, чем при ИЛГ (85,9±25,6 и 57,5±15,3 мм рт. ст.). ДПЖ диастолическое и ДПЖ среднее были достоверно выше у пациентов с ЛАГ-ССД (7,02±5,59 и 28,5±12,7 мм рт. ст.) по сравнению с 0,95±7,6 и 11,7±6,0 мм рт. ст. соответственно. При одинаковых показателях сердечного выброса среднее значение легочного сосудистого сопротивления было выше в группе больных ИЛГ (13,2 и 10,6 ед. Вуда); р<0,005. Форсированная жизненная емкость легких была в пределах нормы в обеих группах, но диффузионная способность легких при ЛАГ-ССД была ниже (46,9±13,5 и 68,5±12,8%). Уровень гемоглобина при ЛАГ-ССД был достоверно меньше (136±22 г/л), чем у пациентов с ИЛГ (149±19 г/л), количество эритроцитов было практически одинаковым (4,9±0,6 и 5,1±0,7 соответственно). Уровень С-реактивного белка у больных ЛАГ-ССД достоверно выше, чем у пациентов с ИЛГ (9,4±15,1 и 1,1±1,0 мг/л); р<0,001. Анализ выживаемости обнаружил достоверное ее снижение в группе ЛАГ-ССД на 2 и 3-м году наблюдения. Расхождение 5-летней выживаемости между группами составило 15%, достоверность была близка к статистически значимой (р=0,06).
Вывод: незначительные различия в клинической картине ЛАГ-ССД и ИЛГ отмечены на фоне существенных отклонений гемодинамических показателей, что оказывает влияние на течение и исход заболевания.
Ключевые слова: идиопатическая легочная гипертензия, легочная гипертензия, ассоциированная с системной склеродермией, выживаемость пациентов с легочной артериальной гипертензией.
Objective: the objective of this report is to compare pts with (SSc-PAH) and (IPAH) based on data of Russian National Registry.
Patients and methods: in the study we included 52 pts with IPAH and 50 with SSc-PAH. There were no differences in functional class (FC). Diagnosis was based on RHC.
Results. At the moment of diagnosis average age of patients with SSc-PAH was 15 year higher (p<0.0001). 6MWD was somewhat more in IPAH group. Borg index was higher in SSc-APAH (4.2±2.1 vs 3.3±1.5); p<0.049. The degree of heart failure (HF) and respiratory failure (RF) did not differ. Dyspnea was the most common among the first symptoms (94% in SSc-PAH and 73% in IPAH). Syncope were observed only in IPAH. In both the diagnosis was verified quite late (in SSc-PAH group in 33 (13; 56.5mts), IPAH – in 30.5 (13.3; 76.3mts). Signs of HF were detected in both groups with equal frequency (76% and 65% respectively). Advanced stage of PAH including symptoms of HF were detected with equal frequency. Only ECHO revealed pericardial effusion more frequently in pts with SSc-PAH (56% vs 35%, p<0.05). Also they had more frequently weight lost (14% vs 2%, p<0.05). Hemodynamics estimation revealed significant haemodynamic differences: sRVP (73.4±19.1 mm Hg) and mPAP (49.1±11.5 mm Hg) in SSc-PAH were significantly lower, than in IPAH (85.9±25.6 mm Hg and 57.5±15.3 mm Hg). dRVP and mRVP were significantly higher in SSc (7.02±5.59 and 28.5±12.7 mm Hg), compared to 0.95±7.6 and 11.7±6.0 mm Hg respectively. Whereas CO values were equal, the average PVR was higher in IPAH (13.2 vs 10.6 WU, р<0.005). RAP levels were equal at both groups. FVC was within normal limits in groups, but DLCO was lower in SSc-PAH group (46.9±13.5% vs 68.5±12.8%). Hb in SSc-APAH group was significantly lower (136±22 g/l vs 149±19 g/l), erythrocyte count was almost same is both groups (4.9±0.6 and 5.1±0.7×1012/l). C-reactive protein in patients with SSc-PAH was higher (9.4±15.1 vs 1.1±1.0 mg/l); p<0.001. Significant decrease in survival during the second and third years of observation was revealed in SSc-PAH group. Difference in 5 years survival between the two groups was 15%, difference was close to statistically significant (p=0.06).
Conclusion: although general clinical appearance of patients with SSc-APAH and IPAH looks similar, there are significant differences in hemodynamic indexes and laboratory signs between the two groups. This factor leads to difference in progress and outcome of the disease.
Key words: idiopathic pulmonary hypertension, pulmonary hypertension associated with systemic sclerosis, the survival rate of patients with pulmonary arterial hypertension.
Цель: провести сравнительную характеристику пациентов с ЛАГ-ССД и ИЛГ по данным Российского национального регистра.
Материалы и методы: 52 больных ИЛГ и 50 – ЛАГ-ССД. Пациенты не различались по функциональному классу (ФК). Средние значения ФК в группах составили 2,7±0,8 и 2,6±0,7 соответственно. Диагноз устанавливался на основании катетеризации правых отделов сердца и легочной артерии.
Результаты. Пациенты с ЛАГ-ССД на момент постановки диагноза были в среднем на 15 лет старше, чем с ИЛГ (р<0,0001). Дистанция 6-минутного теста ходьбы в группе ИЛГ была несколько больше. Индекс Борга был выше в группе ЛАГ-ССД (4,2±2,1 vs 3,3±1,5); р=0,049. Степень недостаточности кровообращения (НК) и дыхательной недостаточности не различались. Самым частым первым симптомом ЛАГ была одышка (94% – при ЛАГ-ССД и 73% – при ИЛГ). Обмороки выявлялись только при ИЛГ (13%). Диагноз ЛАГ в обеих группах устанавливался поздно [при ЛАГ-ССД – через 33 (13; 56,5), при ИЛГ – через 30,5 (13,3; 76,3) мес]. Признаки НК на момент установления диагноза выявлялись с одинаковой частотой как при ЛАГ-ССД, так и ИЛГ (76 и 65% соответственно). Проявления развернутой стадии заболевания, включая признаки НК, выявлялись с одинаковой частотой. Лишь при эхокардиографии у пациентов c ЛАГ-ССД чаще обнаруживался перикардиальный выпот (56 и 35%); р<0,05. Больные с ССД имели достоверно более низкую массу тела (14 и 2%); р<0,05. При оценке центральной гемодинамики обнаружены достоверные различия: давление в правом желудочке (ДПЖ) систолическое (73,4±19,1 мм рт. ст.) и среднее давление в легочной артерии – ДЛАср (49,1±11,5 мм рт. ст.) при ЛАГ-ССД было достоверно ниже, чем при ИЛГ (85,9±25,6 и 57,5±15,3 мм рт. ст.). ДПЖ диастолическое и ДПЖ среднее были достоверно выше у пациентов с ЛАГ-ССД (7,02±5,59 и 28,5±12,7 мм рт. ст.) по сравнению с 0,95±7,6 и 11,7±6,0 мм рт. ст. соответственно. При одинаковых показателях сердечного выброса среднее значение легочного сосудистого сопротивления было выше в группе больных ИЛГ (13,2 и 10,6 ед. Вуда); р<0,005. Форсированная жизненная емкость легких была в пределах нормы в обеих группах, но диффузионная способность легких при ЛАГ-ССД была ниже (46,9±13,5 и 68,5±12,8%). Уровень гемоглобина при ЛАГ-ССД был достоверно меньше (136±22 г/л), чем у пациентов с ИЛГ (149±19 г/л), количество эритроцитов было практически одинаковым (4,9±0,6 и 5,1±0,7 соответственно). Уровень С-реактивного белка у больных ЛАГ-ССД достоверно выше, чем у пациентов с ИЛГ (9,4±15,1 и 1,1±1,0 мг/л); р<0,001. Анализ выживаемости обнаружил достоверное ее снижение в группе ЛАГ-ССД на 2 и 3-м году наблюдения. Расхождение 5-летней выживаемости между группами составило 15%, достоверность была близка к статистически значимой (р=0,06).
Вывод: незначительные различия в клинической картине ЛАГ-ССД и ИЛГ отмечены на фоне существенных отклонений гемодинамических показателей, что оказывает влияние на течение и исход заболевания.
Ключевые слова: идиопатическая легочная гипертензия, легочная гипертензия, ассоциированная с системной склеродермией, выживаемость пациентов с легочной артериальной гипертензией.
________________________________________________
Objective: the objective of this report is to compare pts with (SSc-PAH) and (IPAH) based on data of Russian National Registry.
Patients and methods: in the study we included 52 pts with IPAH and 50 with SSc-PAH. There were no differences in functional class (FC). Diagnosis was based on RHC.
Results. At the moment of diagnosis average age of patients with SSc-PAH was 15 year higher (p<0.0001). 6MWD was somewhat more in IPAH group. Borg index was higher in SSc-APAH (4.2±2.1 vs 3.3±1.5); p<0.049. The degree of heart failure (HF) and respiratory failure (RF) did not differ. Dyspnea was the most common among the first symptoms (94% in SSc-PAH and 73% in IPAH). Syncope were observed only in IPAH. In both the diagnosis was verified quite late (in SSc-PAH group in 33 (13; 56.5mts), IPAH – in 30.5 (13.3; 76.3mts). Signs of HF were detected in both groups with equal frequency (76% and 65% respectively). Advanced stage of PAH including symptoms of HF were detected with equal frequency. Only ECHO revealed pericardial effusion more frequently in pts with SSc-PAH (56% vs 35%, p<0.05). Also they had more frequently weight lost (14% vs 2%, p<0.05). Hemodynamics estimation revealed significant haemodynamic differences: sRVP (73.4±19.1 mm Hg) and mPAP (49.1±11.5 mm Hg) in SSc-PAH were significantly lower, than in IPAH (85.9±25.6 mm Hg and 57.5±15.3 mm Hg). dRVP and mRVP were significantly higher in SSc (7.02±5.59 and 28.5±12.7 mm Hg), compared to 0.95±7.6 and 11.7±6.0 mm Hg respectively. Whereas CO values were equal, the average PVR was higher in IPAH (13.2 vs 10.6 WU, р<0.005). RAP levels were equal at both groups. FVC was within normal limits in groups, but DLCO was lower in SSc-PAH group (46.9±13.5% vs 68.5±12.8%). Hb in SSc-APAH group was significantly lower (136±22 g/l vs 149±19 g/l), erythrocyte count was almost same is both groups (4.9±0.6 and 5.1±0.7×1012/l). C-reactive protein in patients with SSc-PAH was higher (9.4±15.1 vs 1.1±1.0 mg/l); p<0.001. Significant decrease in survival during the second and third years of observation was revealed in SSc-PAH group. Difference in 5 years survival between the two groups was 15%, difference was close to statistically significant (p=0.06).
Conclusion: although general clinical appearance of patients with SSc-APAH and IPAH looks similar, there are significant differences in hemodynamic indexes and laboratory signs between the two groups. This factor leads to difference in progress and outcome of the disease.
Key words: idiopathic pulmonary hypertension, pulmonary hypertension associated with systemic sclerosis, the survival rate of patients with pulmonary arterial hypertension.
Полный текст
Список литературы
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24. Chemla D, Castelain V, Hervé P et al. Haemodynamic evaluation of pulmonary hypertension. Eur Respir J 2002; 20: 1314–31.
25. Fisher MR, Mathai SC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50.
26. Clements PJ, Tan M, McLaughlin VV et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH. Ann Rheum Dis 2012; 71 (2): 249–52.
27. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.
28. Kuhn KP, Byrne DW, Arbogast PG et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 2003; 167: 580–6.
29. Fernandes F, Ramires FJ, Arteaga E et al. Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail 2003; 9: 311–7.
30. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005; 16: 13–8.
31. Hinderliter AL, Willis PW, Long W et al. For the PPH Study Group. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension: primary pulmonary hypertension. Am J Cardiol 1999; 84: 481–4.
32. Miller AJ, Pick R, Johnson PJ. The production of acute pericardial effusion: the effects of various degrees of interference with venous blood and lymph drainage from the heart muscle in the dog. Am J Cardiol 1971; 28: 463–6.
33. Mellins RB, Levine OR, Fishman AP. Effect of systemic and pulmonary venous hypertension on pleural and pericardial fluid accumulation. J Appl Physiol 1970; 29: 564–9.
34. Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 1998; 37: 1320–3.
35. Simeon CP, Armadans L, Fonollosa V et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42: 71–5.
36. Ruiter G, Lankhorst S, Boonstra A et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011; 37 (6): 1386–91.
37. Rhodes CJ, Howard LS, Busbridge M et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: Clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300–9.
38. Ruiter G, Lanser IJ, De Man FS et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53 (2): 285–92.
39. Takeda Y, Takeda Y, Tomimoto S et al. Bilirubin as a prognostic marker in patients with pulmonary arterial hypertension. BMC Pulm Med 2010; 10: 22.
40. Kubo SH, Walter BA, John DH et al. Liver function abnormalities in chronic heart failure. Influence of systemic hemodynamics. Arch Intern Med 1987; 147: 1227–30.
41. Giallourakis CC, Rosenberg PM, Friedman LS. The liver in heart failure. Clin Liver Dis 2002; 6: 947–67.
42. Nagaya N, Uematsu M, Satoh T et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160 (2): 487–92.
43. Leyva F, Anker SD, Godsland IF et al. Uric acid in chronic heart failure: A marker of chronic inflammation. Eur Heart J 1998; 19: 1814–22.
44. Kawut SM, Taichman DB, Archer-Chicko CL et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344–50.
45. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; 35: 989–93.
2. Volkov A.V. Legochnaia arterial'naia gipertenziia pri sistemnykh zabolevaniiakh soedinitel'noi tkani. Nauchno-prakt. revmatologiia. 2015; 1 (53): 69–77. [in Russian]
3. Galiè N, Humbert M, Vachiery JL et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46 (4): 903–75.
4. Fisher MR, Mathai MC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50. Doi: 10.1002/art.22069.
5. Launay D, Sitbon O, Le Pavec J et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoid or sildenafil. Rheumatology 2010: 49 (3): 490–500.
6. LeRoy EC, Black C, Fleischmajer R et. al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15 (2): 202–5.
7. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29 (2): 239–54.
8. Maricq HR, Weinrich MC, Keil JE et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 1989; 32 (8): 998–1006.
9. Mukerjee D, St. George D, Knight C et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford) 2004; 43: 461–6.
10. Hachulla E, De Groote P, Gressin V et al. Itinér AIR-Sclérodermie Study Group. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009; 60 (6): 1831–9.
11. Volkov A.V., Martyniuk T.V., Iudkina N.N. i dr. Vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnoi sklerodermiei. Terapevt. arkh. 2012; 5: 24–8. [in Russian]
12. Launay D, Sitbon O, Hachulla E et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis 2013; 72: 1940–6.
13. Overbeek MJ, Lankhaar JW, Westerhof N et al. Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. Eur Respir J 2008; 31 (6): 1160–6. Doi: 10.1183/09031936.00135407.
14. Tedford RJ, Mudd JO, Girgis RE et al. Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circ Heart Fail 2013; 6 (5): 953–63.
15. Kelemen BW, Mathai SC, Tedford RJ et al. Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes. Pulm Circ 2015; 5 (2): 327–34. Doi: 10.1086/680356.
16. Chung L, Liu J, Parsons L et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010; 138 (6): 1383–94.
17. Volkov A.V., Iudkina N.N., Nikolaeva E.V. i dr. Bozentan: sushchestvennoe uvelichenie prodolzhitel'nosti zhizni patsientov s legochnoi arterial'noi gipertoniei, assotsiirovannoi s sistemnymi revmaticheskimi zabolevaniiami. Terapevt. arkh. 2014; 86 (5): 32–9. [in Russian]
18. Volkov A.V., Nikolaeva E.V., Iudkina N.N. i dr. Vliianie terapii sildenafilom na vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnymi zabolevaniiami soedinitel'noi tkani (rezul'taty prospektivnogo nabliudeniia). Terapevt. arkh. 2015; 87 (11): 62–7. [in Russian]
19. Avdeev S.N., Tsareva N.A., Nekliudova G.V., Chuchalin A.G. Pervyi klinicheskii opyt primeneniia antagonista retseptorov endotelina bozentana u patsientov s legochnoi arterial'noi gipertenziei: rezul'taty godichnogo issledovaniia. Terapevt. arkh. 2013; 85 (3): 38–43. [in Russian]
20. Chazova I.E., Avdeev S.N., Tsareva N.A. i dr. Klinicheskie rekomendatsii po diagnostike i lecheniiu legochnoi gipertonii. Terapevt. arkh. 2014; 9: 4–23. [in Russian]
21. Van den Hoogen F, Khanna D, Fransen J et al. Classification Criteria for Systemic Sclerosis: An ACR-EULAR Collaborative Initiative. Arthritis Rheum 2013; 65 (11): 2737–47.
22. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166: 111–7.
23. Benza RL, Gomberg-Maitland M, Miller DP et al. The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension. Chest 2012; 141 (2): 354–62.
24. Chemla D, Castelain V, Hervé P et al. Haemodynamic evaluation of pulmonary hypertension. Eur Respir J 2002; 20: 1314–31.
25. Fisher MR, Mathai SC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50.
26. Clements PJ, Tan M, McLaughlin VV et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH. Ann Rheum Dis 2012; 71 (2): 249–52.
27. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.
28. Kuhn KP, Byrne DW, Arbogast PG et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 2003; 167: 580–6.
29. Fernandes F, Ramires FJ, Arteaga E et al. Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail 2003; 9: 311–7.
30. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005; 16: 13–8.
31. Hinderliter AL, Willis PW, Long W et al. For the PPH Study Group. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension: primary pulmonary hypertension. Am J Cardiol 1999; 84: 481–4.
32. Miller AJ, Pick R, Johnson PJ. The production of acute pericardial effusion: the effects of various degrees of interference with venous blood and lymph drainage from the heart muscle in the dog. Am J Cardiol 1971; 28: 463–6.
33. Mellins RB, Levine OR, Fishman AP. Effect of systemic and pulmonary venous hypertension on pleural and pericardial fluid accumulation. J Appl Physiol 1970; 29: 564–9.
34. Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 1998; 37: 1320–3.
35. Simeon CP, Armadans L, Fonollosa V et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42: 71–5.
36. Ruiter G, Lankhorst S, Boonstra A et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011; 37 (6): 1386–91.
37. Rhodes CJ, Howard LS, Busbridge M et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: Clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300–9.
38. Ruiter G, Lanser IJ, De Man FS et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53 (2): 285–92.
39. Takeda Y, Takeda Y, Tomimoto S et al. Bilirubin as a prognostic marker in patients with pulmonary arterial hypertension. BMC Pulm Med 2010; 10: 22.
40. Kubo SH, Walter BA, John DH et al. Liver function abnormalities in chronic heart failure. Influence of systemic hemodynamics. Arch Intern Med 1987; 147: 1227–30.
41. Giallourakis CC, Rosenberg PM, Friedman LS. The liver in heart failure. Clin Liver Dis 2002; 6: 947–67.
42. Nagaya N, Uematsu M, Satoh T et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160 (2): 487–92.
43. Leyva F, Anker SD, Godsland IF et al. Uric acid in chronic heart failure: A marker of chronic inflammation. Eur Heart J 1998; 19: 1814–22.
44. Kawut SM, Taichman DB, Archer-Chicko CL et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344–50.
45. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; 35: 989–93.
2. Волков А.В. Легочная артериальная гипертензия при системных заболеваниях соединительной ткани. Научно-практ. ревматология. 2015; 1 (53): 69–77. / Volkov A.V. Legochnaia arterial'naia gipertenziia pri sistemnykh zabolevaniiakh soedinitel'noi tkani. Nauchno-prakt. revmatologiia. 2015; 1 (53): 69–77. [in Russian]
3. Galiè N, Humbert M, Vachiery JL et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46 (4): 903–75.
4. Fisher MR, Mathai MC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50. Doi: 10.1002/art.22069.
5. Launay D, Sitbon O, Le Pavec J et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoid or sildenafil. Rheumatology 2010: 49 (3): 490–500.
6. LeRoy EC, Black C, Fleischmajer R et. al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15 (2): 202–5.
7. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29 (2): 239–54.
8. Maricq HR, Weinrich MC, Keil JE et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 1989; 32 (8): 998–1006.
9. Mukerjee D, St. George D, Knight C et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford) 2004; 43: 461–6.
10. Hachulla E, De Groote P, Gressin V et al. Itinér AIR-Sclérodermie Study Group. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009; 60 (6): 1831–9.
11. Волков А.В., Мартынюк Т.В., Юдкина Н.Н. и др. Выживаемость пациентов с легочной артериальной гипертензией, ассоциированной с системной склеродермией. Терапевт. арх. 2012; 5: 24–8. / Volkov A.V., Martyniuk T.V., Iudkina N.N. i dr. Vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnoi sklerodermiei. Terapevt. arkh. 2012; 5: 24–8. [in Russian]
12. Launay D, Sitbon O, Hachulla E et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis 2013; 72: 1940–6.
13. Overbeek MJ, Lankhaar JW, Westerhof N et al. Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. Eur Respir J 2008; 31 (6): 1160–6. Doi: 10.1183/09031936.00135407.
14. Tedford RJ, Mudd JO, Girgis RE et al. Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circ Heart Fail 2013; 6 (5): 953–63.
15. Kelemen BW, Mathai SC, Tedford RJ et al. Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes. Pulm Circ 2015; 5 (2): 327–34. Doi: 10.1086/680356.
16. Chung L, Liu J, Parsons L et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010; 138 (6): 1383–94.
17. Волков А.В., Юдкина Н.Н., Николаева Е.В. и др. Бозентан: существенное увеличение продолжительности жизни пациентов с легочной артериальной гипертонией, ассоциированной с системными ревматическими заболеваниями. Терапевт. арх. 2014; 86 (5): 32–9. / Volkov A.V., Iudkina N.N., Nikolaeva E.V. i dr. Bozentan: sushchestvennoe uvelichenie prodolzhitel'nosti zhizni patsientov s legochnoi arterial'noi gipertoniei, assotsiirovannoi s sistemnymi revmaticheskimi zabolevaniiami. Terapevt. arkh. 2014; 86 (5): 32–9. [in Russian]
18. Волков А.В., Николаева Е.В., Юдкина Н.Н. и др. Влияние терапии силденафилом на выживаемость пациентов с легочной артериальной гипертензией, ассоциированной с системными заболеваниями соединительной ткани (результаты проспективного наблюдения). Терапевт. арх. 2015; 87 (11): 62–7. / Volkov A.V., Nikolaeva E.V., Iudkina N.N. i dr. Vliianie terapii sildenafilom na vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnymi zabolevaniiami soedinitel'noi tkani (rezul'taty prospektivnogo nabliudeniia). Terapevt. arkh. 2015; 87 (11): 62–7. [in Russian]
19. Авдеев С.Н., Царева Н.А., Неклюдова Г.В., Чучалин А.Г. Первый клинический опыт применения антагониста рецепторов эндотелина бозентана у пациентов с легочной артериальной гипертензией: результаты годичного исследования. Терапевт. арх. 2013; 85 (3): 38–43. / Avdeev S.N., Tsareva N.A., Nekliudova G.V., Chuchalin A.G. Pervyi klinicheskii opyt primeneniia antagonista retseptorov endotelina bozentana u patsientov s legochnoi arterial'noi gipertenziei: rezul'taty godichnogo issledovaniia. Terapevt. arkh. 2013; 85 (3): 38–43. [in Russian]
20. Чазова И.Е., Авдеев С.Н., Царева Н.А. и др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевт. арх. 2014; 9: 4–23. / Chazova I.E., Avdeev S.N., Tsareva N.A. i dr. Klinicheskie rekomendatsii po diagnostike i lecheniiu legochnoi gipertonii. Terapevt. arkh. 2014; 9: 4–23. [in Russian]
21. Van den Hoogen F, Khanna D, Fransen J et al. Classification Criteria for Systemic Sclerosis: An ACR-EULAR Collaborative Initiative. Arthritis Rheum 2013; 65 (11): 2737–47.
22. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166: 111–7.
23. Benza RL, Gomberg-Maitland M, Miller DP et al. The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension. Chest 2012; 141 (2): 354–62.
24. Chemla D, Castelain V, Hervé P et al. Haemodynamic evaluation of pulmonary hypertension. Eur Respir J 2002; 20: 1314–31.
25. Fisher MR, Mathai SC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50.
26. Clements PJ, Tan M, McLaughlin VV et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH. Ann Rheum Dis 2012; 71 (2): 249–52.
27. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.
28. Kuhn KP, Byrne DW, Arbogast PG et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 2003; 167: 580–6.
29. Fernandes F, Ramires FJ, Arteaga E et al. Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail 2003; 9: 311–7.
30. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005; 16: 13–8.
31. Hinderliter AL, Willis PW, Long W et al. For the PPH Study Group. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension: primary pulmonary hypertension. Am J Cardiol 1999; 84: 481–4.
32. Miller AJ, Pick R, Johnson PJ. The production of acute pericardial effusion: the effects of various degrees of interference with venous blood and lymph drainage from the heart muscle in the dog. Am J Cardiol 1971; 28: 463–6.
33. Mellins RB, Levine OR, Fishman AP. Effect of systemic and pulmonary venous hypertension on pleural and pericardial fluid accumulation. J Appl Physiol 1970; 29: 564–9.
34. Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 1998; 37: 1320–3.
35. Simeon CP, Armadans L, Fonollosa V et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42: 71–5.
36. Ruiter G, Lankhorst S, Boonstra A et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011; 37 (6): 1386–91.
37. Rhodes CJ, Howard LS, Busbridge M et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: Clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300–9.
38. Ruiter G, Lanser IJ, De Man FS et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53 (2): 285–92.
39. Takeda Y, Takeda Y, Tomimoto S et al. Bilirubin as a prognostic marker in patients with pulmonary arterial hypertension. BMC Pulm Med 2010; 10: 22.
40. Kubo SH, Walter BA, John DH et al. Liver function abnormalities in chronic heart failure. Influence of systemic hemodynamics. Arch Intern Med 1987; 147: 1227–30.
41. Giallourakis CC, Rosenberg PM, Friedman LS. The liver in heart failure. Clin Liver Dis 2002; 6: 947–67.
42. Nagaya N, Uematsu M, Satoh T et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160 (2): 487–92.
43. Leyva F, Anker SD, Godsland IF et al. Uric acid in chronic heart failure: A marker of chronic inflammation. Eur Heart J 1998; 19: 1814–22.
44. Kawut SM, Taichman DB, Archer-Chicko CL et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344–50.
45. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; 35: 989–93.
________________________________________________
2. Volkov A.V. Legochnaia arterial'naia gipertenziia pri sistemnykh zabolevaniiakh soedinitel'noi tkani. Nauchno-prakt. revmatologiia. 2015; 1 (53): 69–77. [in Russian]
3. Galiè N, Humbert M, Vachiery JL et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46 (4): 903–75.
4. Fisher MR, Mathai MC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043–50. Doi: 10.1002/art.22069.
5. Launay D, Sitbon O, Le Pavec J et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoid or sildenafil. Rheumatology 2010: 49 (3): 490–500.
6. LeRoy EC, Black C, Fleischmajer R et. al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15 (2): 202–5.
7. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29 (2): 239–54.
8. Maricq HR, Weinrich MC, Keil JE et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 1989; 32 (8): 998–1006.
9. Mukerjee D, St. George D, Knight C et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford) 2004; 43: 461–6.
10. Hachulla E, De Groote P, Gressin V et al. Itinér AIR-Sclérodermie Study Group. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009; 60 (6): 1831–9.
11. Volkov A.V., Martyniuk T.V., Iudkina N.N. i dr. Vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnoi sklerodermiei. Terapevt. arkh. 2012; 5: 24–8. [in Russian]
12. Launay D, Sitbon O, Hachulla E et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis 2013; 72: 1940–6.
13. Overbeek MJ, Lankhaar JW, Westerhof N et al. Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. Eur Respir J 2008; 31 (6): 1160–6. Doi: 10.1183/09031936.00135407.
14. Tedford RJ, Mudd JO, Girgis RE et al. Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circ Heart Fail 2013; 6 (5): 953–63.
15. Kelemen BW, Mathai SC, Tedford RJ et al. Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes. Pulm Circ 2015; 5 (2): 327–34. Doi: 10.1086/680356.
16. Chung L, Liu J, Parsons L et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010; 138 (6): 1383–94.
17. Volkov A.V., Iudkina N.N., Nikolaeva E.V. i dr. Bozentan: sushchestvennoe uvelichenie prodolzhitel'nosti zhizni patsientov s legochnoi arterial'noi gipertoniei, assotsiirovannoi s sistemnymi revmaticheskimi zabolevaniiami. Terapevt. arkh. 2014; 86 (5): 32–9. [in Russian]
18. Volkov A.V., Nikolaeva E.V., Iudkina N.N. i dr. Vliianie terapii sildenafilom na vyzhivaemost' patsientov s legochnoi arterial'noi gipertenziei, assotsiirovannoi s sistemnymi zabolevaniiami soedinitel'noi tkani (rezul'taty prospektivnogo nabliudeniia). Terapevt. arkh. 2015; 87 (11): 62–7. [in Russian]
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Авторы
Н.Н.Юдкина1, Э.Г.Валеева2, И.Н.Таран2, Е.В.Николаева1, В.М.Парамонов2, И.А.Курмуков1, З.С.Валиева2, О.А.Архипова2, Т.В.Мартынюк2, А.В.Волков1, Е.Л.Насонов1, И.Е.Чазова*2
1 ФГБНУ Научно-исследовательский институт ревматологии им. В.А.Насоновой. 115522, Россия, Москва, Каширское ш., д. 34а;
2 Институт клинической кардиологии им. А.Л.Мясникова ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*chazova@hotmail.com
1 V.A.Nasonova Research Institute of Rheumatology. 115522, Russian Federation, Moscow, Kashirskoe sh., d. 34a;
2 A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation. 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a
*chazova@hotmail.com
1 ФГБНУ Научно-исследовательский институт ревматологии им. В.А.Насоновой. 115522, Россия, Москва, Каширское ш., д. 34а;
2 Институт клинической кардиологии им. А.Л.Мясникова ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*chazova@hotmail.com
________________________________________________
1 V.A.Nasonova Research Institute of Rheumatology. 115522, Russian Federation, Moscow, Kashirskoe sh., d. 34a;
2 A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation. 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a
*chazova@hotmail.com
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