Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up.

Autor: Vilela VS; Rheumatology Discipline, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Dias MM; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Salgado ÂA; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., da Silva BRA; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Lopes AJ; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Bessa EJC; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Bruno LP; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., da Costa CH; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil., Levy RA; Rheumatology Discipline, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil.; Immunology and Specialty Medicine, GSK, 1250 Collegville Rd, Collegeville, PA, 19426, USA., Rufino R; Cardiology Discipline, Thorax Diseases Department, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil. rrufino.uerj@gmail.com.
Jazyk: angličtina
Zdroj: BMC pulmonary medicine [BMC Pulm Med] 2021 Jul 29; Vol. 21 (1), pp. 251. Date of Electronic Publication: 2021 Jul 29.
DOI: 10.1186/s12890-021-01618-z
Abstrakt: Background: Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population.
Methods: A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated.
Results: Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20- < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively.
Conclusions: Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better.
Trial Registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.
(© 2021. The Author(s).)
Databáze: MEDLINE
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