Assessment of pulmonary arterial stiffness in patients with systemic sclerosis without overt pulmonary hypertension

Abstract

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and complicate most systemic diseases. Systemic sclerosis (SSc), represents the leading cause of connective tissue disease (CTD) associated with PAH. Although SSc is a rare disease, it is associated with higher morbidity and early mortality than other rheumatological diseases due to developing SSc-associated interstitial pulmonary disease (ILD) and/or pulmonary arterial hypertension (PAH). The impact of the early diagnosis on the prognosis is evident. In this context, in our study, we aimed to investigate the early changes in pulmonary vascular bed by measuring pulmonary arterial stiffness (PAS) in SSc patients without overt PAH. Sixty-two SSc patients and fifty-eight gender and age-matched, healthy subjects enrolled in this cross-sectional observational study. SSc patients were evaluated in terms of disease duration and severity. Modified rodnan skin score (mRSS) was calculated as disease severity index. Echocardiographic parameters were assessed and compared to the control group. Right ventricular (RV) diameters, systolic pulmonary artery pressure (sPAP), and right ventricle myocardial performance index (RV-MPI) were significantly higher in the SSc group compared to the control group (p < 0.05). Tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) were significantly lower in the SSc group compared to the control group (p < 0.05). PAS value (25.5 +/- 9.2 kHz/ms vs. 18.1 +/- 7.4 kHz/ms, p < 0.001) was significantly higher in the SSc group than in the control group. A statistically significant positive correlation relationship was detected between the PAS value and CRP, ESR, disease duration, mRSS. According to these results, in SSc patients, PAS as an inexpensive and easily applicable echocardiographic method might serve as a marker of early detection of PAH.