Podemos predecir la gravedad de la hipertensión arterial pulmonar en pacientes con esclerodermia

a b s t r a c t 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 Echocardiographyand 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 Sclero- derma 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 DLCOand the increase in TRV are negative predictor factor of PAH which,