Pulmonary arterial hypertension associated with systemic scleroderma
E. K. Popova,
N. S. Arkhipova,
N. V. Ilyin,
A. D. Asekritov,
E. A. Ignatiev,
D. V. Solovyova,
M. G. Neustroeva,
I. O. Popov https://doi.org/10.30629/0023-2149-2021-99-1-68-74
Abstract
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.
About the Authors
E. K. Popova
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
Elena K. Popova — MD, PhD, assistant professor, Department of Public Health and Health Care, General Hygiene and Bioethics
677000, Yakutsk
N. S. Arkhipova
Republican Hospital № 1 — National Center of Medicine
Russian Federation
Russian Federation
677010, Yakutsk
N. V. Ilyin
Republican Hospital № 1 — National Center of Medicine
Russian Federation
Russian Federation
677010, Yakutsk
A. D. Asekritov
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
677000, Yakutsk
E. A. Ignatiev
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
677000, Yakutsk
D. V. Solovyova
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
677000, Yakutsk
M. G. Neustroeva
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
677000, Yakutsk
I. O. Popov
Medical Institute of North-Eastern Federal University named M.K. Ammosov
Russian Federation
Russian Federation
677000, Yakutsk
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Review
For citations:
Popova E.K., Arkhipova N.S., Ilyin N.V., Asekritov A.D., Ignatiev E.A., Solovyova D.V., Neustroeva M.G., Popov I.O. Pulmonary arterial hypertension associated with systemic scleroderma. Clinical Medicine (Russian Journal). 2021;99(1):68-74. (In Russ.) https://doi.org/10.30629/0023-2149-2021-99-1-68-74
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