rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2019-445-451rsp-2758Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL RESEARCHЭндотелиальная дисфункция и нейрогуморальная регуляция артериального давления у больных системной склеродермиейENDOTHELIAL DYSFUNCTION AND NEUROHUMORAL REGULATION OF BLOOD PRESSURE IN PATIENTS WITH SYSTEMIC SCLEROSISШилкинаН. П.ShilkinaN. P.

Ярославль

Yaroslavl

shilkin39@mail.ruЮнонинИ. Е.YunoninI. E.

Ярославль

Yaroslavl

СтоляроваС. А.StolyarovaS. A.

Ярославль

Yaroslavl

ВиноградовА. А.VinogradovA. A.

Ярославль

Yaroslavl

ФГБОУ ВО «Ярославский государственный медицинский университет»РоссияYaroslavl State Medical University, Ministry of Health of RussiaRussian FederationГБКУЗ ЯО «Центральная городская больница»РоссияYaroslavl State Medical University, Ministry of Health of RussiaRussian Federation201916092019574445451Copyright © Шилкина Н.П., Юнонин И.Е., Столярова С.А., Виноградов А.А., 20192019Шилкина Н.П., Юнонин И.Е., Столярова С.А., Виноградов А.А.Shilkina N.P., Yunonin I.E., Stolyarova S.A., Vinogradov A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2758

Цель исследования – изучить влияние дисфункции эндотелия, катехоламинов и ренина на суточный профиль артериального давления (АД) у больных системной склеродермией (ССД).Материал и методы. У 25 пациентов, страдающих ССД, проведено определение количества десквамированных эндотелиоцитов в крови методом J. Hladovec (1978), уровня эндотелина-1, адреналина, норадреналина и ренина плазмы крови методом иммуноферментного анализа. Всем пациентам выполнено суточное мониторирование АД с расчетом индекса времени, суточного индекса, величины и скорости утреннего подъема АД, а также суточной вариабельности АД.Результаты и обсуждение. Артериальная гипертензия выявлена у 8 (32%) пациентов с ССД. У всех больных ССД отмечены признаки дисфункции эндотелия, о чем свидетельствуют значительные различия показателей активации эндотелия по сравнению с контрольной группой: средний уровень эндотелина-1 составил 5,8±2,3 и 0,48±0,21 фмоль/мл (р<0,05), количество десквамированных эндотелиоцитов – 4,50 [3,00; 7,00] и 2,10 [1,00; 3,20] • 104/л соответственно (р<0,05). Содержание адреналина и норадреналина при ССД было значимо выше, чем в контроле. Отмечались положительные корреляции дисфункции эндотелия и степени повышения АД. Выявлено негативное влияние дисфункции эндотелия на суточный профиль АД с наличием патологических типов night-peaker и non-dipper.Заключение. В патогенезе развития клинических симптомов ССД, включая артериальную гипертензию, прослеживаются два механизма – эндотелиальная дисфункция и активация симпатоадреналовой системы.

Objective: to investigate the effect of endothelial dysfunction, catecholamines, and renin on the diurnal blood pressure (BP) profile in patients with systemic sclerosis (SS).Subjects and methods. Twenty-five patients with SS underwent determination of the blood count of desquamated endotheliocytes by the method described by J. Hladovec (1978), the plasma levels of endothelin-1 (ET-1), adrenaline, norepinephrine, and renin by enzyme immunoassay. All the patients underwent 24-hour BP monitoring with calculating the time index, daily index, the magnitude and rate of a morning rise in BP, as well as its daily variability.Results and discussion. Hypertension was detected in 8 (32%) patients with SS. All the patients with SS showed signs of endothelial dysfunction, as evidenced by considerable differences in endothelial activation measures compared with the control group: the mean level of ET-1 was 5.8±2.3 and 0.48±0.21 fmol/ml (p<0.05); the number of desquamated endotheliocytes was 4.50 [3.00; 7.00] and 2.10 [1.00; 3.20] • 104/l, respectively (p<0.05). The levels of adrenaline and norepinephrine in SS were significantly higher than those in the control. There were positive correlations between endothelial dysfunction and the degree of an increase in BP. Endothelial dysfunction was found to have a negative impact on the diurnal BP profile in the presence of pathological types of night-peaker and non-dipper. Conclusion. Two mechanisms, such as endothelial dysfunction and sympathoadrenal activation, are responsible for the pathogenesis of clinical symptoms of SS, including hypertension.

системная склеродермиядисфункция эндотелияартериальная гипертензиянейрогуморальная регуляцияsystemic sclerosisendothelial dysfunctionhypertensionneurohumoral regulationReferencesГусева НГ. Системная склеродермия: ранняя диагностика и прогноз. Научно-практическая ревматология. 2007;45(1):39-45Guseva NG. Systemic sclerosis: early diagnosis and prognosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2007;45(1):39-45 (In Russ.).Ананьева ЛП. Новые классификационные критерии системной склеродермии (лекция). Научно-практическая ревматология. 2013;51(5):539-44 doi: 10.14412/1995-4484-2013-1546Ananyeva LP. New classification criteria for scleroderma systematica (a lecture). Nauchno- Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2013;51(5):539-44 (In Russ.). doi: 10.14412/1995-4484-2013-1546Конева ОА, Овсянникова ОБ, Старовойтова МН и др. Определение чувствительности новых критериев системной склеродермии на российской популяции пациентов. Научно-практическая ревматология. 2015;53(4):361-6 doi: 10.14412/1995-4484-2015-361-366Koneva OA, Ovsyannikova OB, Starovoitova MN, et al. Aassessment of thesensitivity of new criteria for systemic sclerosis in Russian patient population. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2015; 53(4):361-6 (In Russ.). doi: 10.14412/1995-4484-2015-361-366Wollheim FA. Classification of systemic sclerosis: visions and reality. Rheumatology (Oxford). 2005;44:1212-6. doi: 10.1093/rheumatology/keh671Koneva OA, Ovsyannikova OB, Starovoitova MN, et al. Aassessment of thesensitivity of new criteria for systemic sclerosis in Russian patient population. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2015; 53(4):361-6 (In Russ.). doi: 10.14412/1995-4484-2015-361-366Van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification Criteria For Systemic Sclerosis: an American College of Rheumatology / European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424Wollheim FA. Classification of systemic sclerosis: visions and reality. Rheumatology (Oxford). 2005;44:1212-6. doi: 10.1093/rheumatology/keh671Furue M, Mitoma C, Mitoma H, et al. Pathogenesis of systemic sclerosis-current concept and emerging treatments. Immunol Res. 2017 Aug;65(4):790-7. doi: 10.1007/s12026-017-8926-yWollheim FA. Classification of systemic sclerosis: visions and reality. Rheumatology (Oxford). 2005;44:1212-6. doi: 10.1093/rheumatology/keh671Ананьева ЛП, Александрова ЕН. Аутоантитела при системной склеродермии: спектр, клинические ассоциации и прогностическое значение. Научно-практическая ревматология. 2016;54(1):86-99 doi: 10.14412/1995-4484-2016-86-99Wollheim FA. Classification of systemic sclerosis: visions and reality. Rheumatology (Oxford). 2005;44:1212-6. doi: 10.1093/rheumatology/keh671Бутусова СВ, Шилкина НП. Ранняя диагностика поражения эндотелия у пациентов с ревматоидным артритом. Врач. 2014;(5):67-9Van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification Criteria For Systemic Sclerosis: an American College of Rheumatology / European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424Klimek E, Skalska A. Differential associations of inflammatory and endothelial biomarkers with disease activity in rheumatoid arthritis of short duration. Mediators Inflamm. 2014;2014:ID681635. doi: 10.1155/2014/681635Van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification Criteria For Systemic Sclerosis: an American College of Rheumatology / European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424Routledge FS, Hinderliter AL. Endothelial function in postmenopausal women with nighttime systolic hypertension. Menopause. 2015;8:857-63. doi: 10.1097/GME.0000000000000405Van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification Criteria For Systemic Sclerosis: an American College of Rheumatology / European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424Small HY, Migliatino S, Czesnikiewicz-Guzik M, Guzik TJ. Hypertension: Focus on autoimmunity and oxidative stress. Free Radic Biol Med. 2018 Sep;125:104-15. doi: 10.1016/j.freeradbiomed.2018.05.085Furue M, Mitoma C, Mitoma H, et al. Pathogenesis of systemic sclerosis-current concept and emerging treatments. Immunol Res. 2017 Aug;65(4):790-7. doi: 10.1007/s12026-017-8926-yWeber T, Wassertheurer S. Relationship between 24-hour ambulatory central blood pressure and left ventricular mass – a prospective multicenter study. J Hypertens. 2016;34 Suppl. 1):Abstract Book: 210-11.Furue M, Mitoma C, Mitoma H, et al. Pathogenesis of systemic sclerosis-current concept and emerging treatments. Immunol Res. 2017 Aug;65(4):790-7. doi: 10.1007/s12026-017-8926-yРеброва НВ, Анисимова ЕА, Саркисова ОЛ и др. Реактивность сосудов головного мозга у больных ревматоидным артритом в сочетании с артериальной гипертонией и без нее. Терапевтический архив. 2015;87(4):24-9 doi: 10.17116/terarkh201587424-29Furue M, Mitoma C, Mitoma H, et al. Pathogenesis of systemic sclerosis-current concept and emerging treatments. Immunol Res. 2017 Aug;65(4):790-7. doi: 10.1007/s12026-017-8926-yChen CT, Li Y. Association between ambulatory systolic blood pressure during the day and asymptomatic intracranial arterial stenosis. J Hypertens. 2014;63:61-7. doi: 10.1161/HYPERTENSIONAHA.113.01838Ananyeva LP, Aleksandrova EN. Autoantibodies in scleroderma systematica: Spectrum, clinical associations, and prognostic value. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2016;54(1):86-99 (In Russ.). doi: 10.14412/1995-4484-2016-86-99Черемухина ЕО, Ананьева ЛП, Новикова ДС, Алекперов РТ. Вариабельность сердечного ритма при системной склеродермии. Клиническая фармакология и терапия. 2015;24(1):61-5Ananyeva LP, Aleksandrova EN. Autoantibodies in scleroderma systematica: Spectrum, clinical associations, and prognostic value. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2016;54(1):86-99 (In Russ.). doi: 10.14412/1995-4484-2016-86-99Хусаинова ДК, Трегулов ЮЭ, Салихов ИГ и др. Эндотелиальная дисфункция и артериальная гипертензия у больных ревматоидным артритом. Научно-практическая ревматология. 2006;44(3):27-32Ananyeva LP, Aleksandrova EN. Autoantibodies in scleroderma systematica: Spectrum, clinical associations, and prognostic value. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2016;54(1):86-99 (In Russ.). doi: 10.14412/1995-4484-2016-86-99Старовойтова МН, Десинова ОВ. Терапия дигитальных язв у больных системной склеродермией. Научно-практическая ревматология. 2018;56(6):777-81 doi: 10.14412/1995-4484-2018-777-781Butusova SV, Shilkina NP. Early diagnosis of endothelial lesions in patients with rheumatoid arthritis. Vrach. 2014;(5):67-9 (In Russ.).Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Butusova SV, Shilkina NP. Early diagnosis of endothelial lesions in patients with rheumatoid arthritis. Vrach. 2014;(5):67-9 (In Russ.).Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Butusova SV, Shilkina NP. Early diagnosis of endothelial lesions in patients with rheumatoid arthritis. Vrach. 2014;(5):67-9 (In Russ.).Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Klimek E, Skalska A. Differential associations of inflammatory and endothelial biomarkers with disease activity in rheumatoid arthritis of short duration. Mediators Inflamm. 2014;2014:ID681635. doi: 10.1155/2014/681635Алекперов РТ, Ананьева ЛП, Черкасова МВ. Клинические ассоциации сосудистого эндотелиального фактора роста и его рецептора 2-го типа при системной склеродермии. Научно-практическая ревматология. 2018;56(5):569-73 doi: 10.14412/1995-4484-2018-569-573Klimek E, Skalska A. Differential associations of inflammatory and endothelial biomarkers with disease activity in rheumatoid arthritis of short duration. Mediators Inflamm. 2014;2014:ID681635. doi: 10.1155/2014/681635Ребров АП, Патрикеева ДА, Захарова НБ и др. Диагностическое значение определения факторов ангиогенеза и показателей цитокинового состава в сыворотке крови и в моче у пациентов с системной склеродермией. Терапевтический архив. 2014;(5):19-25Klimek E, Skalska A. Differential associations of inflammatory and endothelial biomarkers with disease activity in rheumatoid arthritis of short duration. Mediators Inflamm. 2014;2014:ID681635. doi: 10.1155/2014/681635Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Routledge FS, Hinderliter AL. Endothelial function in postmenopausal women with nighttime systolic hypertension. Menopause. 2015;8:857-63. doi: 10.1097/GME.0000000000000405Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Routledge FS, Hinderliter AL. Endothelial function in postmenopausal women with nighttime systolic hypertension. Menopause. 2015;8:857-63. doi: 10.1097/GME.0000000000000405Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Routledge FS, Hinderliter AL. Endothelial function in postmenopausal women with nighttime systolic hypertension. Menopause. 2015;8:857-63. doi: 10.1097/GME.0000000000000405Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Small HY, Migliatino S, Czesnikiewicz-Guzik M, Guzik TJ. Hypertension: Focus on autoimmunity and oxidative stress. Free Radic Biol Med. 2018 Sep;125:104-15. doi: 10.1016/j.freeradbiomed.2018.05.085Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Small HY, Migliatino S, Czesnikiewicz-Guzik M, Guzik TJ. Hypertension: Focus on autoimmunity and oxidative stress. Free Radic Biol Med. 2018 Sep;125:104-15. doi: 10.1016/j.freeradbiomed.2018.05.085Шилкина НП, Савина ЖЕ, Юнонин ИЕ. Гиперрениновые формы артериальной гипертензии при системной красной волчанке и их коррекция. Научно-практическая ревматология. 2017;55(2, прил.1):139-40Small HY, Migliatino S, Czesnikiewicz-Guzik M, Guzik TJ. Hypertension: Focus on autoimmunity and oxidative stress. Free Radic Biol Med. 2018 Sep;125:104-15. doi: 10.1016/j.freeradbiomed.2018.05.085Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Weber T, Wassertheurer S. Relationship between 24-hour ambulatory central blood pressure and left ventricular mass – a prospective multicenter study. J Hypertens. 2016;34 Suppl. 1):Abstract Book: 210-11.Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Weber T, Wassertheurer S. Relationship between 24-hour ambulatory central blood pressure and left ventricular mass – a prospective multicenter study. J Hypertens. 2016;34 Suppl. 1):Abstract Book: 210-11.Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Weber T, Wassertheurer S. Relationship between 24-hour ambulatory central blood pressure and left ventricular mass – a prospective multicenter study. J Hypertens. 2016;34 Suppl. 1):Abstract Book: 210-11.Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Rebrova NV, Anisimova EA, Sarkisova OL, et al. Cerebrovascular reactivity in patients with rheumatoid arthritis concurrent with and without hypertension. Terapevticheskiy arkhiv. 2015;87(4):24-9 (In Russ.). doi: 10.17116/terarkh201587424-29Кляус НА, Симакова МА, Маслянский АЛ, Моисеева ОМ. Поиск клинических предикторов легочной гипертензии у пациентов с системной склеродермией. Научно-практическая ревматология. 2018;56(5):586-90 doi: 10.14412/1995-4484-2018-586-590Rebrova NV, Anisimova EA, Sarkisova OL, et al. Cerebrovascular reactivity in patients with rheumatoid arthritis concurrent with and without hypertension. Terapevticheskiy arkhiv. 2015;87(4):24-9 (In Russ.). doi: 10.17116/terarkh201587424-29Corallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yRebrova NV, Anisimova EA, Sarkisova OL, et al. Cerebrovascular reactivity in patients with rheumatoid arthritis concurrent with and without hypertension. Terapevticheskiy arkhiv. 2015;87(4):24-9 (In Russ.). doi: 10.17116/terarkh201587424-29Волков АВ, Мартынюк ТВ, Юдкина НН и др. Первый российский опыт применения ингибитора рецепторов эндотелина 1 траклира у пациентов с легочной артериальной гипертензией, ассоциированной с системными заболеваниями соединительной ткани. Научно-практическая ревматология. 2011;49(6):22-7 doi: 10.14412/1995-4484-2011-515Chen CT, Li Y. Association between ambulatory systolic blood pressure during the day and asymptomatic intracranial arterial stenosis. J Hypertens. 2014;63:61-7. doi: 10.1161/HYPERTENSIONAHA.113.01838Невская ТА, Ананьева ЛП, Быковская СЮ и др. Нарушения ангиогенеза и клеточные методы их коррекции при системной склеродермии. В кн.: V съезд ревматологов России: Сб. мат. съезда (тезисы). Москва; 2009Chen CT, Li Y. Association between ambulatory systolic blood pressure during the day and asymptomatic intracranial arterial stenosis. J Hypertens. 2014;63:61-7. doi: 10.1161/HYPERTENSIONAHA.113.01838Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Chen CT, Li Y. Association between ambulatory systolic blood pressure during the day and asymptomatic intracranial arterial stenosis. J Hypertens. 2014;63:61-7. doi: 10.1161/HYPERTENSIONAHA.113.01838Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Cheremukhina EO, Anan'eva LP, Novikova DS, Alekperov RT. Heart rate variability in systemic scleroderma. Klinicheskaya Farmakologiya i Terapiya. 2015;24(1):61-5 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Khusainova DK, Tregulov YuE, Salikhov IG, et al. Endothelial dysfunction and arterial hypertension in patients with rheumatoid arthritis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2006;44(3):27-32 (In Russ.).Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Starovoitova MN, Desinova OV. Therapy for digital ulcers in patients with systemic scleroderma. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(6):777-81 (In Russ.). doi: 10.14412/1995-4484-2018-777-781Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rhеumatol Rep. 2013;15:303. doi: 10.1007/s11926-012-0303-1Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Silva I, Teixeira A, Oliveira J, et al. Endothelial dysfunction, microvascular damadge and ischemic peripheral vasculopathy in systemic sclerosis. Clin Hemorheol Microcirc. 2017;66 (2):117-30. doi: 10.3233/CH-150044Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Jung C, Drummer K, Oelzner P, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud's phenomenon as detected by functional imaging. Clin Hemorheol Microcirc. 2015;61(3):549-57. doi: 10.3233/CH-151956Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Alekperov RT, Ananyeva LP, Cherkasova MV. Clinical associations of vascular endothelial growth factor and its type 2 receptor in systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):569-73 (In Russ.). doi: 10.14412/1995-4484-2018-569-573Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Rebrov AP, Patrikeeva DA, Zakharova NB, et al. Diagnostic value of determining angiogenesis factors and cytokine composition indicators in serum and urine in patients with systemic scleroderma. Terapevticheskii Arkhiv. 2014;(5):19-25 (In Russ.).Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Cipriani P, Marrelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmune Rev. 2011;10(10):641-6. doi: 10.1016/j.autrev.2011.04.019Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67. doi: 10.1172%2FJCI31139Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Manetti M, Romano E, Rosa I, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis. 2017 May;76(5):924-34. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Romano E, Rosa I, Fioretto BS, et al. A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and nervous systems. Clin Exp Rheumatol. 2019;11 [Epub ahead of print].Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Dessein PH, Joffe BI, Metz RM, et al. Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Arthritis Rheum. 1994 Jan;37(1):67-74.Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Shilkina NP, Savina ZhE, Yunonin IE. Hyperrenin forms of arterial hypertension in systemic lupus erythematosus and their correction. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2017;55(2 Suppl.1):139-40 (In Russ.).Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Woodworth TG, Suliman YA, Li W, et al. Sleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-91. doi: 10.1038/nrnerh.2016.124Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Si Ahmed-Bouali D, Bouali F, Haddoum F, et al. Hypertension in scleroderma: A vital emergency. Ann Cardiol Angeiol. 2015;64(3):192-8. doi: 10.1016/ancard.2015.04.009Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;0:1-12. doi: 10.1136/annrheumdis-2016-209909Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Korn JH, Mayes M, Matucci-Cerenic M, for the RAPIDS-1 study group. Digital ulcers in systemic sclerosis. Prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rhеum. 2004;50:3985-93. doi: 10.1002/art.20676Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Klyaus NA, Simakova MA, Maslyansky AL, Moiseeva OM. Search for clinical predictors of pulmonary hypertension in patients with systemic sclerosis. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2018;56(5):586-90 (In Russ.). doi: 10.14412/1995-4484-2018-586-590Corallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yCorallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yCorallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yCorallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yCorallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yCorallo C, Cutolo M, Kahaleh B, et al. Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. Arthritis Res Ther. 2016 Oct 6;18(1):228. doi: 10.1186/s13075-016-1122-yVolkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Volkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Volkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Volkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Volkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Volkov AV, Martynyuk TV, Yudkina NN, et al. The first Russian experience with the endothelin 1 receptor inhibitor traclir used in patients with pulmonary hypertension associated with systemic connective tissue diseases. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(6):22-7 (In Russ.). doi: 10.14412/1995-4484-2011-515Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Nevskaya TA, Anan'eva LP, Bykovskaya SYu, et al. Angiogenesis disorders and cellular methods for their correction in systemic scleroderma. In: V s"ezd revmatologov Rossii: Sb. mat. s"ezda (tezisy) [V congress of rheumatologists of Russia: Collection of materials of the congress (theses)]. Moscow; 2009 (In Russ.).Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1Pokeerbux MR, Giovannelli J, Dauchet L, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and metaanalysis of the literature. Arthritis Res Ther. 2019;21:86 doi: 10.1186/s13075-019-1867-1

The authors declare that there are no conflicts of interest present.