¿Podemos predecir la gravedad de la hipertensión arterial pulmonar en pacientes con esclerodermia? / Can we predict the severity of pulmonary hypertension in patients with scleroderma?

Objetivos. Describir las características clínico-biológicas de pacientes con esclerodermia (ES) e hipertensión arterial pulmonar (HTAP). Establecer la relación entre las pruebas funcionales respiratorias (PFR), la ecocardiografía Doppler (eco-Doppler) y la gravedad de la HTAP. Material y métodos. Estudio retrospectivo de pacientes con diagnóstico de ES seguidos en un centro de tercer nivel. Se les realizó un estudio protocolizado con analítica completa y pruebas complementarias; se estimó la presión arterial pulmonar sistólica (PAPs), la velocidad de reflujo de la válvula tricúspide (VRT), la difusión de monóxido de carbono (DLCO) y la capacidad vital forzada (CVF), por medio de la eco-Doppler y la PFR. Resultados. Se incluyó a 331 pacientes, de los cuales 68 (20,5%) tenían HTAP. El subtipo de ES más prevalente fue la limitada. Se calculó el coeficiente de correlación de Pearson a las siguientes variables: CVF-PAPs CVF/DLCO-PAPs, DLCO-PAP y VRT-PAPs, observándose una asociación lineal moderada significativa en la relación DLCO-PAPs y con VRT-PAP. Se constataron 29 fallecimientos, 12 relacionados a la HTAP. El tiempo medio entre el diagnóstico de HTAP y la muerte fue de 1,8 años. Conclusiones. La disminución de la DLCO y el aumento de la VRT son factores predictores de HTAP, que al mismo tiempo condiciona un peor pronóstico en los pacientes con ES (AU)

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 (AU)

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

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.