Abstract
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.
Highlights
The hallmarks of systemic sclerosis (SSc) are microvascular disorders, autoimmune disturbances, and fibrosis of the skin and internal organs [1]
Of the 30 SSc patients with New York Heart Association (NYHA) functional class II who underwent cardiovascular magnetic resonance (CMR), 22 (73.3%) had myocarditis, and 20 (66.7%) were given steroid treatment as per protocol (2 were withdrawn from the study because of uncontrolled inflammatory myopathy and needed high-dose steroid and immunosuppressive therapy)
Notwithstanding, according to our current study findings, we found that low stroke volume and high heart rate in SSc patients with myocarditis was suggestive of an expected improvement after steroid therapy
Summary
The hallmarks of systemic sclerosis (SSc) are microvascular disorders, autoimmune disturbances, and fibrosis of the skin and internal organs [1]. Functional (reversible) microvascular disorder can lead to structural (permanent) microvascular occlusion [14], and in turn to focal myocardial ischemia myocardial fibrosis. Such pathological sequelae contribute to ventricular dysfunction, ranging from asymptomatic diastolic or systolic dysfunction, and clinically overt heart failure [6, 12]. Moderate-dose steroid therapy may improve myocarditis in SSc. A proportion of patients died due to cardiac complications during treatment, those with high hs-cTnT, high NT-proBNP, and impaired LVEF. A proportion of patients died due to cardiac complications during treatment, those with high hs-cTnT, high NT-proBNP, and impaired LVEF
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