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Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: Potential utility of immunosuppressive therapy in cardiac damage progression
Abstract
Objectives Scleroderma heart disease is a major risk of death in systemic sclerosis (SSc). Mechanisms underlying myocardial damage are still unclear. We performed an extensive study of SSc patients with recent-onset symptoms for heart disease and examined the efficacy of immunosuppressive therapy. Methods A cohort of 181 SSc patients was enrolled. Of these, 7 patients newly developed clinical symptoms of heart disease (heart failure, chest pain, and palpitation); all of them showed mild but persistent increase in cardiac enzymes. These patients underwent Holter ECG, 2D-echocardiography, perfusional scintigraphy, delayed-enhancement-cardiac magnetic resonance (DE-CMR), coronary angiography, and endomyocardial biopsy. Patients were treated for at least 12 months and followed-up for 5 years. Results Ventricular ectopic beats (VEBs) were found in 4 patients, wall motion abnormalities in 3, pericardial effusion in 6, and DE in CMR in 6 with T2-hyperintensity in 2. In all patients, histology showed upregulation of endothelium adhesion molecules and infiltration of activated T lymphocytes, with (acute/active myocarditis in 6) or without (chronic/borderline myocarditis in 1) myocyte necrosis. Parvovirus B19 genome was detected in 3. None showed occlusion of coronary arteries or microvessels. Compared with SSc controls, these patients more often had early disease, skeletal myositis, c-ANCA/anti-PR3 positivity, VEBs, pericardial effusion, and systolic and/or diastolic dysfunction. Immu…
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Systemic sclerosis and systemic lupus erythematosus have been linked to virus-negative lymphocytic myocarditis.
“Autoimmune conditions such as systemic sclerosis and systemic lupus erythematosus have been linked to virus-negative lymphocytic myocarditis.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [153]
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Sorted by citations
Myocarditis: An Interleukin-1-Mediated Disease?
De Luca, G., Cavalli, G., Campochiaro, C., Tresoldi, M., & Dagna, L. (2018) · Frontiers in Immunology · doi:10.3389/fimmu.2018.01335 · Cited by 79
Autoimmune Myocarditis, Old Dogs and New Tricks.
Won, T., Song, E. J., Kalinoski, H. M., Moslehi, J. J., & Čiháková, D. (2024) · Circulation Research · doi:10.1161/circresaha.124.323816 · Cited by 51
Metadata
- Authors
- Pieroni, M., De Santis, M., Zizzo, G., Bosello, S., Smaldone, C., Campioni, M., De Luca, G., Laria, A., Meduri, A., Bellocci, F., Bonomo, L., Crea, F., & Ferraccioli, G.
- Affiliations
- Università Cattolica del Sacro Cuore (IT)
- Corresponding author
- Ferraccioli, Gianfranco
- ORCID
- Venue
- Seminars in Arthritis and Rheumatism
- Publisher
- Elsevier BV
- Published
- 2014-02
- Volume
- 43
- Issue
- 4
- Pages
- 526-535
- ISSN
- 0049-0172 (ISSN-L)
- OpenAlex
- W2132151198
- Cited by
- 158
- References
- 0
- Citations by year
- 2026: 7 · 2025: 9 · 2024: 17 · 2023: 14 · 2022: 14 · 2021: 9 · 2020: 13 · 2019: 23 · 2018: 11 · 2017: 19 · 2016: 12 · 2015: 8 · 2014: 2
- FWCI
- 5.49
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Seminars in Arthritis and Rheumatism (publishedVersion)
- PubMed (publishedVersion)
- Florence Research (University of Florence) (submittedVersion)
- Archivio Istituzionale della Ricerca - Università Vita-Salute San Raffaele (submittedVersion)
- Topics
- Health Sciences › Medicine › Rheumatology › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Infectious Diseases › Viral Infections and Immunology Research
- Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
- Keywords
- systemic sclerosis, myocarditis, immunosuppressive therapy, premature ventricular contractions, pericardial effusion, parvovirus B19, anti-PR3 ANCA, sudden cardiac death
- MeSH terms
- Adult, Aged, Female, Humans, Immunosuppressive Agents, Male, Middle Aged, Myocarditis, Scleroderma, Systemic, Treatment Outcome, Disease Progression
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