rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2018-474-485rsp-2594Research ArticleПРОГРАММА НЕПРЕРЫВНОГО ПОСЛЕДИПЛОМНОГО ОБРАЗОВАНИЯ ВРАЧЕЙPOSTGRADUATE PROGRAM OF CONTINUING MEDICAL EDUCATIONЛегочная артериальная гипертензия при системных заболеваниях соединительной ткани: современное состояние проблемыPULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC CONNECTIVE TISSUE DISEASES: THE CURRENT STATE OF THE PROBLEMВолковА. В.VolkovA. V.

зав. лабораторией инструментальной и УЗ-диагностики ФГБНУ НИИР им. В.А.Насоновой, канд. мед. наук

sandyvlk@yahoo.comМартынюкТ. В.MartynyukT. V.

руководитель отдела легочной гипертензии и заболеваний сердца ФГБУ НМИЦ кардиологии, профессор кафедры кардиологии ФДПО РНИМУ им. Н.И. Пирогова, докт. мед. наук

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», МоскваРоссияV.A. Nasonova Research Institute of Rheumatology, Moscow, RussiaRussian FederationФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава России, Москва; ФГБОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, МоскваРоссияNational Medical Research Center of Cardiology, Ministry of Health of Russia, Moscow; N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, MoscowRussian Federation201808092018564474485Copyright © Волков А.В., Мартынюк Т.В., 20182018Волков А.В., Мартынюк Т.В.Volkov A.V., Martynyuk T.V.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/2594

Легочная артериальная гипертензия (ЛАГ) – комплексная, мультидисциплинарная проблема современной медицины. В основе заболевания лежит поражение сосудов микроциркуляторного русла, приводящее к снижению в них кровотока, повышению легочного сосудистого сопротивления и, как исход, правожелудочковой сердечной недостаточности и смерти. ЛАГ также может ассоциироваться с системными заболеваниями соединительной ткани (СтЗСТ), в частности с системной склеродермией (ССД), выступая в качестве одного из проявлений заболевания. Так же как идиопатическая легочная гипертензия, ЛАГ-СтЗСТ является орфанным заболеванием: по данным ФГБНУ НИИР им. В.А. Насоновой, встречаемость ЛАГ при ССД составляет 5,2%, при системной красной волчанке – 0,3%, а при смешанном заболевании соединительной ткани – 7,4%. Низкая распространенность обусловливает позднюю диагностику, что всегда ассоциируется с плохим ответом на терапию и неблагоприятным прогнозом. Цель лекции – ознакомить ревматологов с возможностями диагностики и лечения этого редкого, но прогностически неблагоприятного проявления СтЗСТ.

Pulmonary arterial hypertension (PAH) is a complex, multidisciplinary problem of modern medicine. The basis for the disease is a microcirculatory lesion in the vessels, which leads to a decrease in their blood flow, to increased pulmonary vascular resistance and, as an outcome, right ventricular failure, and death. PAH may also be associated with systemic connective tissue diseases (SCTDs), in particular with systemic sclerosis (SS), as one of the manifestations of the disease. As well as idiopathic pulmonary hypertension, PAH-SCTDs is an orphan disease: according to the data of the V.A. Nasonova Research Institute of Rheumatology, the incidence of PAH is 5.2% in SS, 0.3% in systemic lupus erythematosus, and 7.4% in mixed connective tissue disease. Low prevalence is responsible for late diagnosis, which is always associated with a poor therapy response and poor prognosis. The purpose of the lecture is to acquaint rheumatologists with the possibilities of diagnosis and treatment of this rare, but prognostic severe manifestation of SCTDs.

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