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. 2023 Apr 14;18(1):83.
doi: 10.1186/s13023-023-02699-1.

The clinical characteristics and outcomes of patients with systemic sclerosis with myocardial involvement

Huilin He #  1 , Jinzhi Lai #  2 , Jiaxin Zhou  1 , Yong Hou  1 , Dong Xu  3 , Mengtao Li  1 , Xiaofeng Zeng  1
Affiliations

The clinical characteristics and outcomes of patients with systemic sclerosis with myocardial involvement

Huilin He et al. Orphanet J Rare Dis. .

Abstract

Background: Myocardial involvement (MI) is the primary cause of death in patients with systemic sclerosis (SSc). We analyzed patients with SSc and MI to identify their characteristics and outcome.

Method: We retrospectively collected data from SSc patients with MI admitted to Peking Union Medical College Hospital between January 2012 and May 2021. SSc patients without MI were randomly selected as controls after matching age and gender at a ratio of 1:3.

Results: In total, 21 SSc patients (17 females) with MI were enrolled. The mean age at SSc onset was 42.3 ± 15.1 years old. Compared with controls, myositis (42.9% vs. 14.3%, P = 0.014) and elevation of CK (33.3% vs. 4.8%, P = 0.002) were more common in patients with MI. Of the 7 patients without cardiovascular symptoms, 3 /5 showed elevations in cardiac troponin-I (cTnI), 6 showed elevations of N-terminal brain natriuretic peptide (NT-proBNP). Eleven patients were followed up for a median period of 15.5 months and four patients developed newly occurring left ventricular ejection fraction (LVEF) < 50%.

Conclusion: One third of SSc patients with MI were asymptomatic. Regular monitoring of CTnI, NT-proBNP and echocardiography is helpful for the diagnosis of MI during the early stages. Its prognosis is poor.

Keywords: Clinical features; Follow-up; Myocardial involvement; Systemic sclerosis; Treatment.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

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