Pulmonary arterial hypertension associated with systemic scleroderma

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.