Related Experiment Video
Updated: Oct 26, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years
Verônica Silva Vilela1, Marcio Macri Dias2, Ângelo Antunes Salgado2
1Rheumatology Discipline, State University of Rio de Janeiro, Hospital Universitário Pedro Ernesto, Third Floor, Boulevard 28 de Setembro 77, Rio de Janeiro, 20551-031, Brazil.
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.
Related Concept Videos
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
Mitral Stenosis II: Clinical features and Diagnostic Tests
Hypertension III: Clinical Manifestations and Diagnostic Studies

