Demographic and clinical differences between idiopathic and scleroderma-related pulmonary arterial hypertension: Russian National Registry analysis

• Main Authors: N N Yudkina, E G Valeeva, I N Taran, E V Nikolaeva, V M Paramonov, I A Kurmukov, Z S Valieva, O A Arkhipova, T V Martynyuk, A V Volkov, E L Nasonov, I E Chazova
• Format: Article
• Language: Russian
• Published: IP Morozov P.V. 2016-06-01
• Series: Системные гипертензии
• Subjects: idiopathic pulmonary hypertension; pulmonary hypertension associated with systemic sclerosis; the survival rate of patients with pulmonary arterial hypertension
• Online Access: https://syst-hypertension.ru/2075-082X/article/viewFile/29142/pdf

Pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) and idiopathic pulmonary arterial hypertension (IPAH) belong to group I in the clinical classification of PH, but there is evidence for significant differences in their survival due to current therapy. Objective: the objective of this report is to compare pts with (SSc-PAH) and (IPAH) based on data of Russian National Registry. Patients and methods: in the study we included 52 pts with IPAH and 50 with SSc-PAH. There were no differences in functional class (FC). Diagnosis was based on RHC. Results. At the moment of diagnosis average age of patients with SSc-PAH was 15 year higher (p