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Background: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), and is associated with a poor prognosis. SSc-pHI has been historically related to myocardial fibrosis and vascular damage; however, myocardial inflammation is increasingly reported and can be non-invasively detected by novel cardiac magnetic resonance (CMR) techniques. Therapeutic strategies to treat SSc-pHI remain elusive. Objectives: to evaluate the efficacy of immunosuppressive therapy on CMR features and clinical outcomes in patients with CMR-proven SSc-pHI. Methods: we retrospectively analyzed data from SSc patients with pHI 1 confirmed by CMR, who start or modify immunosuppressive therapy as indication for the newly diagnosed pHI and who had a follow-up CMR after 6 to 18 months. Patients were recruited from two national referral centers for SSc. All patients underwent a comprehensive baseline evaluation of disease characteristics and organ involvement, particularly cardiac involvement, including: recording of signs and symptoms of heart disease, serum levels of high-sensitive troponin T and brain natriuretic peptide (BNP) or NT-proBNP, inflammatory markers, standard 12-leads ECG, 24h ECG Holter, echocardiography and CMR at baseline and during follow-up. A p value Results: 35 patients with SSc-pHI (females 77.1%; median age 59 46-64 years; anti-topoisomerase-I positivity 48.6%; diffuse cutaneous subset 34.3%) were enrolled (Table 1). The majority of patients (74.3%) had signs of active myocardial inflammation at baseline CMR, i.e. edema at short-tau inversion recovery (STIR) images and/or increase of T2 mapping; late gadolinium enhancement (LGE) areas were detected in 6 (17.4%) patients, while extra-cellular volume (ECV) and T1 mapping increase in 23 (65.7%) patients each (Table 2). Mycophenolate mofetil was started (up to 2000 mg per day) in 15 patients (42.9%) or increased (up to 3000 mg per day) in 7 (20.0%) cases; 7 patients (20.0%) received rituximab, 3 patients (8.6%) azathioprine (combined with prednisone in 1 case), while intravenous cyclophosphamide (combined with pulse steroids), subcutaneous tocilizumab and hydroxychloroquine were started in one patient each. No other patients received steroids. The median duration of immunosuppressive therapy was 12.0 6.0 – 15.5 months. At the follow-up CMR (performed after a median time 12.0 6.5 – 16.0 months), signs of active myocardial inflammation were detectable in 14 patients (40%) (p=0.003), and STIR images suggestive for edema were still present in only 5 cases (14.3%) (p=0.002). A consistently significant reduction of T2 mapping (from 53.0 49.0–55.0 to 51.0 50.0–54.0, p Conclusion: our study shows that immunosuppression is a therapeutic strategy which can effectively treat newly diagnosed SSc-pHI. Immunosuppression may thus curb signs of myocardial inflammation at CMR, significantly reducing cardiac enzymes, inflammatory markers and overall clinical burden. Larger prospective randomized studies are needed to confirm these data. REFERENCES: 1 Bruni C, et al. J Scleroderma Relat Disord. 2022 Feb;7(1):24-32. doi: 10.1177/23971983211053246. Acknowledgements: NIL. Disclosure of Interests: None declared.
Luca et al. (Sat,) studied this question.