Can we predict the severity of pulmonary hypertension in patients with scleroderma?

Abstract

Objectives: To describe the clinical-biological characteristics of patients with scleroderma (SSc) and pulmonary artery hypertension (PAH). To establish the relationship between pulmonary functional tests (PFT), Doppler echocardiography (ECHO) and the severity of the PAH.

Material and methods: Retrospective study of patients with scleroderma treated at a tertiary center. All participants received a protocol study, which included a complete analysis and additional tests: Doppler Echocardiography and pulmonary functional tests (PFT) with carbon monoxide diffusing capacity (DLCO).

Results: Overall, 331 patients were treated, including 68 (20.5%) with PAH. The limited subtype of Scleroderma was the most prevalent. The Person's correlation coefficient was used for the following variables: FVC-sPAP, FVC/DLCO-sPAP, DLCO-sPAP and TRV-sPAP, showed a significant moderate linear association in the relationship DLCO-sPAP and TRV-sPAP. 29 deaths occurred, with 12 of them related to PAH. The median time between the PAH diagnosis and death was 1.8 years.

Conclusions: The decrease in DLCO and the increase in TRV are negative predictor factor of PAH which, at the same time, means a worsening prognosis for patients with Scleroderma.