Prognosis variables to overall mortality in patients with systemic sclerosis: pilot cohort study

It is widely acknowledged that patients with systemic sclerosis (SSc) experience elevated rates of cardiovascular mortality and morbidity over the course of their lives.

The aim of this exploratory pilot study is to examine the prognostic variables for all-cause mortality detected in patients with SSc detected by nuclear imaging and cardiac CT.

Prospective cohort study. During a period of 4 years (2012 to 2016), 62 (age 56.8 ± 12.9 years, men 12.9%) patients with SSc referred from the Medicine Service for a cardiological evaluation. These patients underwent gated Single-Photon Emission Computed Tomography Myocardial Perfusion Imaging (gSPECT-MPI) stress-rest and also underwent coronary computed tomography angiography (CCTA). Cox regression analysis (STATA 18, StataCorp, College Station, TX, USA) was employed for statistical analyses to evaluate independent predictive variables, with adjustments made for age and gender.

During a long follow-up of 7.6 ± 2.3 years post gSPECT and CCTA, 12 deaths (9 non-cardiac deaths and 3 cardiac deaths) (0.025 exitus/patients/years) was observed. In the univariate Cox regression analysis, previous cardiac event (myocardial infarction, heart failure, unstable angina, coronary revascularization, syncope) (HR: 6.1, p=0.005), coronary artery disease (stenosis > 10%) (HR:8.2, p=0.003), significant coronary artery disease (CAD) ≥ 50% (HR: 3.9, p=0.036), calcium score ≥ 200 AU (HR: 3.2, p=0.045), METs ≤ 5 (HR: 5.3, p=0.007), cold test resistance ≤ 1 minute (HR: 10, p=0.027), summed thickening score at rest (HR: 1.12, p=0.016) and diastolic dysfunction (TPFR: Time to Peak Filling Rate > 250 mseg) (HR: 4.9, p=0.010), were significant. All these variables were used for multivariate Cox analysis, and the final predictive model for all-cause mortality adjusted by age and gender (Harrell’s = 0.905, Somers’ D = 0.809) was (Results Table):

In this pilot cohort study with long follow-up in patients with SSc a significant CAD, the exercise capacity, the cold test resistance and the diastolic disfunction emerged as the most crucial variables in predicting all-cause mortality, demonstrating good accuracy.