Cardiac evaluation in patients with scleroderma_results from screening of pulmonary hypertension in systemic lupus erythermatosus (SOPHIE) study
Authors: Zhou M , Siu C.W 
Published: November 2020

Abstract

Background
Pulmonary hypertension (PH) and cardiac involvement were considered as the important prognostic factors in patients with scleroderma. The purpose of this study is to investigate the PH prevalence and related cardiovascular events in patients with scleroderma who were enrolled for screening of PH in systemic lupus erythermatosus (SOPHIE) study.

Methods
One hundred and ninety-four patients with scleroderma were enrolled in this study. Clinical characteristics were collected, all of patients were received echocardiography for screening of the probability of PH using tricuspid regurgitation jet velocity and other echocardiographic “PH” signs. Additionally, Blood test, B type natriuretic peptide (BNP), serum autoantibodies, and 6 minutes walk test, as well as electrocardiograph, were performed in those patients.

Results
Forty (20.6%) patients were divided into intermediate/ high probability PH group (IHPH), 154 Patients were divided into none/ low probability PH group (NLPH). Patients in IHPH group were older compared to NLPH group and has a higher proportion of males (20% VS 7.8%.NLPH), dermatomyositis (10% VS 1.9%.NLPH), and stage 3 chronic kidney disease (7.5% VS 1.3%.NLPH), with a shorter 6MWT distance (390.2±108.3 VS 444.9±88.5.NLPH). B-type natriuretic peptide (BNP) level (181.4±323.8 VS 51.1±61.8. NLPH) and the proportion of BNP level larger than 100 pg/ml (40% VS 10.3%.NLPH) were increased in IHPH group. No significant difference was observed in serum autoantibodies including anti-Scl-70, anti-centromere, anti-RNP, and antiphospholipid antibody between two groups. Thirty-six (18.6%) patients occurred arrythmias, 3 patients (1.6%) with atrial fibrillation and all belong to the IHPH group, patients in IHPH group presented a higher proportion of premature atrial complex (7.5% VS 1.0%.NLPH) and sinus tachycardia (5% VS 0.6%.NLPH), no significant difference was observed in arrythmias in terms of atrial-ventricular blockage or ventricular conduction blockage. Forty patients (20.6%) occurred abnormal electrical activities, the main difference between two groups were lie in T wave abnormality (20% vs 3.9%. NLPH) and P wave high voltage (7.5% VS 0%.NLPH). Receiver operating characteristic (ROC) curve revealed that BNP larger than 100pg/ml to predict PH with sensitivity (52.9%) and specificity (88.2%). Multiple logistic regression analysis indicates age (OR, 1.1; 95% CI, 1.0, 1.10), BNP (OR, 1.0; 95% CI, 1.0, 1.02), diastolic blood pressure (OR, 1.0; 95% CI, 0.91, 0.98) significantly affected right ventricular systolic pressure.

Conclusions
Patients with scleroderma belonged to IHPH group are relatively older than that of NLPH group and manifested with a higher proportion of males, dermatomyositis, more severely chronic kidney disease, arrhythmias, and abnormal electrical activities. Increased age, BNP levels, and diastolic blood pressure can serve as the predicting factors for pulmonary hypertension in patients with scleroderma.
Figure 1

Funding Acknowledgement
Type of funding source: None