Prevalence of high probability of pulmonary hypertension in early systemic sclerosis. Description of a cohort of patients

Abstract

Introduction

Systemic sclerosis is an autoimmune disease characterized by skin and internal organ fibrosis. In the early stages of the disease, complications such as pulmonary hypertension may manifest. The aim of this study was to evaluate the frequency of high probability of pulmonary hypertension and associated factors in a cohort of patients with early systemic sclerosis or systemic sclerosis without cutaneous involvement.

Methods

Retrospective cohort study. Patients from the rheumatology outpatient clinic of the Hospital Universitario Nacional between 2017 and 2023 were included. Patients were categorized into early systemic sclerosis and systemic sclerosis without cutaneous involvement. The classification of high probability of pulmonary hypertension was based on echocardiographic features, pulmonary anatomy, lung function, and clinical context.

Results

Seventy-one patients with early systemic sclerosis and 51 patients with systemic sclerosis without cutaneous involvement were included in the analysis. Ninety six percent of patients were women, with an average age of 57 years; the most frequent symptoms were related to the gastrointestinal tract. The most common positive autoantibodies were anticentromere antibodies. The prevalence of high probability of pulmonary hypertension was 26%. High probability of group 1 pulmonary hypertension was the most frequent. No factors associated with high probability of pulmonary hypertension were identified.

Conclusions

Studies on patients in early stages of systemic sclerosis are scarce in our setting. High probability of pulmonary hypertension can be common in the early stages of systemic sclerosis. It could be useful to implement screening strategies for pulmonary hypertension in the early stages of systemic sclerosis.