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

Abstract

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

Keywords

• idiopathic pulmonary hypertension
• pulmonary hypertension associated with systemic sclerosis
• the survival rate of patients with pulmonary arterial hypertension