clinmedКлиническая медицинаClinical Medicine (Russian Journal)0023-21492412-1339ООО «Медицинское информационное агентство»10.30629/0023-2149-2021-99-1-68-74clinmed-183Research ArticleЗАМЕТКИ И НАБЛЮДЕНИЯ ИЗ ПРАКТИКИNOTES AND OBSERVATIONS FROM PRACTICEЛегочная артериальная гипертензия, ассоциированная с системной склеродермиейPulmonary arterial hypertension associated with systemic sclerodermahttps://orcid.org/0000-0002-9338-1644ПоповаЕ. К.PopovaE. K.

Попова Елена Капитоновна — кандидат медицинских наук, доцент кафедры пропедевтической и факультетской терапии с эндокринологией и ЛФК

677000, Якутск

Elena K. Popova — MD, PhD, assistant professor, Department of Public Health and Health Care, General Hygiene and Bioethics

677000, Yakutsk

ecapopova@yandex.ruhttps://orcid.org/0000-0002-6433-3424АрхиповаН. С.ArkhipovaN. S.

677010, Якутск

677010, Yakutsk

ИльинН. В.IlyinN. V.

677010, Якутск

677010, Yakutsk

https://orcid.org/0000-0002-3042-722XАсекритовА. Д.AsekritovA. D.

677000, Якутск

677000, Yakutsk

https://orcid.org/0000-0001-8815-6824ИгнатьевЕ. А.IgnatievE. A.

 

677000, Якутск

677000, Yakutsk

https://orcid.org/0000-0003-4496-6707СоловьеваД. В.SolovyovaD. V.

677000, Якутск

677000, Yakutsk

https://orcid.org/0000-0002-7738-7253НеустроеваМ. Г.NeustroevaM. G.

677000, Якутск

677000, Yakutsk

https://orcid.org/0000-0002-0876-561XПоповИ. О.PopovI. O.

677000, Якутск

677000, Yakutsk

Медицинский институт ФГАОУ ВО «Северо-Восточного федерального университета им. М.К. Аммосова» Минобрнауки РоссииРоссияMedical Institute of North-Eastern Federal University named M.K. AmmosovRussian FederationГАУ Республики Саха (Якутия) «Республиканская больница № 1 — Национальный центр медицины» Минздрава РоссииРоссияRepublican Hospital № 1 — National Center of MedicineRussian Federation2021040620219916874Copyright © Попова Е.К., Архипова Н.С., Ильин Н.В., Асекритов А.Д., Игнатьев Е.А., Соловьева Д.В., Неустроева М.Г., Попов И.О., 20212021Попова Е.К., Архипова Н.С., Ильин Н.В., Асекритов А.Д., Игнатьев Е.А., Соловьева Д.В., Неустроева М.Г., Попов И.О.Popova E.K., Arkhipova N.S., Ilyin N.V., Asekritov A.D., Ignatiev E.A., Solovyova D.V., Neustroeva M.G., Popov I.O.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.clinmedjournal.com/jour/article/view/183

Легочная артериальная гипертензия (ЛАГ) относится к орфанным заболеваниям, характеризующимся повышением легочного сосудистого сопротивления (ЛСС). ЛАГ является труднодиагностируемой патологией ввиду неспецифичности первых симптомов. Без ранней диагностики и лечения прогноз ЛАГ крайне неблагоприятен, так при системной склеродермии 60% больных умирают в первые 2 года. В Республике Саха (Якутия) в настоящее время насчитывается 38 пациентов с ЛАГ, при этом наблюдается тенденция к увеличению их количества. С этим связана необходимость проведения научных исследований пациентов с ЛАГ, проживающих на территории Республики Саха (Якутия). Представлено клиническое наблюдение в течение 1,5 года за пациенткой 45 лет с ЛАГ, ассоциированной с системной склеродермией, которое демонстрирует трудности в дифференциальной диагностике ЛАГ, позднее начало применения ЛАГ-специфической фармакотерапии. В статье рассматривается эффективность применения оригинальных ЛАГ-специфических лекарственных препаратов и их дженериков.

Pulmonary arterial hypertension (PAH) is an orphan disease characterized by an increase in pulmonary vascular resistance (PVR). PAH is a pathology, difficult to diagnose due to the non-specificity of its first strokes. The prognosis of PAH is extremely unfavorable without early diagnosis and treatment, as with systemic scleroderma, 60% of patients die in the first 2 years. In the Republic of Sakha (Yakutia), there are currently 38 patients with PAH, and a tendency towards an increase in their number is noted. The necessity to conduct scientific research on PAH patients living in the Republic of Sakha (Yakutia) is urgent due to the fact. The article represents a 1.5-year clinical observation of a 45-year-old patient with PAH associated with systemic scleroderma, which demonstrates difficulties in the differential diagnosis of PAH, late initiation of PAH-specific pharmacotherapy. The article discusses the efficiency of the original PAH-specific drugs use, and their generics.

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