Clinical and laboratory profiles of 136 systemic sclerosis patients with and without echocardiographically detected pulmonary hypertension

Abstract

This study demonstrates that SSc patients with PH detected by echocardiography had characteristic clinical and laboratory features. More specific treatment addressing these aspects should be offered to improve the curative effect of therapy in SSc-PH patients.

Premature atherosclerosis in systemic sclerosis patients: Its relation to disease parameters and to traditional risk factors

“…Esophageal involvement was determined by barium swallow and echocardiography was done to detect left ventricular ejection fraction and pulmonary hypertension. Pulmonary hypertension was present if the systolic pulmonary artery pressure (sPAP) exceeded 40 mmHg by echocardiography …”

Serum Biomarkers in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

“…Other SSc-specific autoantibodies (i.e., anti-topoisomerase I and anti-RNA polymerase III) have not shown an association with PAH [ 92 ]. Anti-ribonucleoprotein (RNP) antibodies have been found to be associated with PH [ 95 ] and PAH in SSc patients [ 96 , 97 , 98 ]: anti-U1RNP have been detected in 9% patients with SSc-PAH, and approximately 22% of anti-U1RNP-positive SSc patients were affected by PAH. Anti-U3RNP was detected in about 24% of SSc-PAH patients, whereas PAH was found in 25% of SSc patients with positive anti-U3RNP, suggesting that this autoantibody may play a role in the development of PAH [ 92 ].…”

“…Although anti-U1RNP antibodies were not clearly associated with SSc-PAH, they appear to have a strong association with PAH in SLE patients. In a case–control study comparing 84 SLE-PAH patients and 160 SLE patients without PAH, anti-U1RNP antibodies were independently associated with severe PAH and more active SLE [ 97 ]. In a multicentric longitudinal study carried out in a cohort of 3624 consecutive SLE patients, a 10-year probability-predictive nomogram for the development of PAH, including clinical variables and three autoantibodies (anti-RNP, anti-SSA and anti-SSB) was developed, confirming the close association between the anti-RNP antibodies and PAH in SLE [ 102 ].…”