Background. Myocarditis is reported in systemic sclerosis (SSc); however, treatment options and outcomes are limited. Our objective was to define cardiac outcomes after moderate-dose steroid therapy in SSc patients with myocarditis. Method. An open-label study was conducted among SSc patients with myocarditis—as defined by cardiovascular magnetic resonance (CMR), disease onset <5 years, and a NYHA functional class ≥II. All enrolled patients received prednisolone (30 mg/d) which would be tapered off by week 24, and CMR was followed up at the end of treatment. Results. A total of 20 SSc patients were enrolled which 12 patients completed the study. At week 24, 8 of the 12 cases experienced improvement of myocarditis. Compared to those with no improvement, these 8 patients had significantly longer disease duration ( p = 0.03 ), higher heart rate at baseline ( p = 0.049 ) and week 24 ( p = 0.04 ), lower left ventricular (LV) and right ventricular (RV) stroke volume at baseline ( p = 0.002 and p = 0.01 ) and week 24 ( p = 0.01 and p = 0.02 ), and lower LV and RV cardiac output at week 24 ( p = 0.01 and p = 0.01 ). Four cases died during follow-up (3 due to cardiac complications, 1 due to renal crisis). The two who died from heart failure had very high NT-prohormone-brain natriuretic peptide (NT-proBNP) and impaired LV ejection fraction (LVEF), and the one who died from arrhythmia had very high sensitivity of cardiac Troponin-T (hs-cTnT). Conclusions. Moderate-dose steroid therapy may improve myocarditis in SSc. A proportion of patients died due to cardiac complications during treatment, particularly those with high hs-cTnT, high NT-proBNP, and impaired LVEF. This trial is registered with NCT03607071.

Background: Cardiac magnetic resonance imaging (cardiac MRI) has high sensitivity and specificity for differentiating cardiac fibrosis from inflammation. There is no data on the clinical and laboratory association or prediction of myocardial inflammation in systemic sclerosis-a major organ involvement in systemic sclerosis (SSc). Objectives:Our aim was to ascertain the clinical and laboratory associations with myocardial inflammation in SSc patients as detected by cardiac MRI.