rpcardioRational Pharmacotherapy in CardiologyРациональная Фармакотерапия в Кардиологии1819-64462225-3653«SILICEA-POLIGRAF» LLC10.20996/1819-6446-2014-10-1-73-78rpcardio-145Research ArticlePOINT OF VIEWТОЧКА ЗРЕНИЯCHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION AND PROBLEMS OF RARE AND INTERDISCIPLINARY DISEASEХРОНИЧЕСКАЯ ТРОМБОЭМБОЛИЧЕСКАЯ ЛЕГОЧНАЯ ГИПЕРТЕНЗИЯ И ПРОБЛЕМЫ РЕДКОГО И МЕЖДИСЦИПЛИНАРНОГО ЗАБОЛЕВАНИЯШостакН. А.ShostakN. A.

Шостак Надежда Александровна — доктор медицинских наук, профессор, заведующая кафедрой факультетской терапии им. акад. А.И. Нестерова лечебного факультета РНИМУ

aaklimenko@yandex.ruКлименкоА. А.KlimenkoA. A.

Клименко Алеся Александровна — кандидат медицинских наук,  доцент кафедры факультетской терапии им. акад. А.И. Нестерова лечебного факультета

aaklimenko@yandex.ruДемидоваН. А.DemidovaN. A.

Демидова Наталья Александровна — ассистент кафедры факультетской терапии им. акад. А.И. Нестерова лечебного факультета

aaklimenko@yandex.ruРоссийский национальный исследовательский медицинский университет им. Н.И. Пирогова, МоскваРоссияPirogov Russian National Research Medical University, MoscowRussian Federation2014230920151017378Copyright © Shostak N.A., Klimenko A.A., Demidova N.A., 20152015Шостак Н.А., Клименко А.А., Демидова Н.А.Shostak N.A., Klimenko A.A., Demidova N.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.rpcardio.online/jour/article/view/145

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare life-threatening disease with a prevalence of 2 cases per 100000 population. CTEPH is a chronic, progressive disease characterized by high disability and mortality rates in young and middle-aged people, often with underlying genetic and autoimmune thrombophilic disorders. The need for pathogenetic therapy with orphan drugs that can slow the progression of the disease is supported.

Хроническая тромбоэмболическая легочная гипертензия (ХТЛГ) является редким жизнеугрожающим заболеванием с распространенностью 2 случая на 100000  населения. ХТЛГ – это хроническое прогрессирующее заболевание, характеризующееся высокой степенью инвалидизации и высокой смертностью у лиц молодого и среднего возраста, часто имеющее в основе генетические и аутоиммунные тромбофилические нарушения. Приводится обоснование необходимости применения патогенетической терапии орфанными препаратами, позволяющими замедлить прогрессирование заболевания.

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