Cited by 158Systemic Sclerosis and Related DiseasesViral Infections and Immunology ResearchEosinophilic Disorders and Syndromes

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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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Research works printed but not linked: 59

  1. Kahan, 2006, Primary myocardial involvement in systemic sclerosis, Rheumatology, 45, iv14, 10.1093/rheumatology/kel312 · DOI
  2. Steen, 2000, Severe organ involvement in systemic sclerosis with diffuse scleroderma, Arthritis Rheum, 43, 2437, 10.1002/1529-0131(200011)43:11<2437::AID-ANR10>3.0.CO;2-U · DOI
  3. Ferri, 2002, Systemic sclerosis: demographic, clinical, and serologic features and survival in 1012 Italian patients, Medicine (Baltimore), 81, 139, 10.1097/00005792-200203000-00004 · DOI
  4. Scussel-Lonzetti, 2002, Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival, Medicine (Baltimore), 81, 154, 10.1097/00005792-200203000-00005 · DOI
  5. Ioannidis, 2005, Mortality in systemic sclerosis: an international meta-analysis of individual patient data, Am J Med, 118, 2, 10.1016/j.amjmed.2004.04.031 · DOI
  6. De Santis, 2012, Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease, Clin Respir J, 6, 9, 10.1111/j.1752-699X.2010.00228.x · DOI
  7. Bulkley, 1976, Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction, Circulation, 53, 483, 10.1161/01.CIR.53.3.483 · DOI
  8. Alexander, 1986, Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis, Ann Intern Med, 105, 661, 10.7326/0003-4819-105-5-661 · DOI
  9. Kahan, 1986, Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis, N Engl J Med, 314, 1397, 10.1056/NEJM198605293142201 · DOI
  10. Steen, 1996, Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis, Arthritis Rheum, 39, 677, 10.1002/art.1780390421 · DOI
  11. Duboc, 1991, The effect of nifedipine on myocardial perfusion and metabolism in systemic sclerosis. A positron emission tomographic study, Arthritis Rheum, 34, 198, 10.1002/art.1780340211 · DOI
  12. Vignaux, 2005, Evaluation of the effect of nifedipine upon myocardial perfusion and contractility using cardiac magnetic resonance imaging and tissue Doppler echocardiography in systemic sclerosis, Ann Rheum Dis, 64, 1268, 10.1136/ard.2004.031484 · DOI
  13. Kobayashi, 2009, Cardiac magnetic resonance imaging with pharmacological stress perfusion and delayed enhancement in asymptomatic patients with systemic sclerosis, J Rheumatol, 36, 106, 10.3899/jrheum.080377 · DOI
  14. James, 1974, De subitaneis mortibus. VIII. Coronary arteries and conduction system in scleroderma heart disease, Circulation, 50, 844, 10.1161/01.CIR.50.4.844 · DOI
  15. Kahan, 1985, Decreased coronary reserve in primary scleroderma myocardial disease, Arthritis Rheum, 28, 637, 10.1002/art.1780280607 · DOI
  16. Montisci, 2003, Detection of early impairment of coronary flow reserve in patients with systemic sclerosis, Ann Rheum Dis, 62, 890, 10.1136/ard.62.9.890 · DOI
  17. Fernandes, 2003, Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study, J Card Fail, 9, 311, 10.1054/jcaf.2003.51 · DOI
  18. Tzelepis, 2007, Pattern and distribution of myocardial fibrosis in systemic scleroderma, a delayed enhanced MRI study, Arthritis Rheum, 56, 3827, 10.1002/art.22971 · DOI
  19. Hachulla, 2009, Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients, Ann Rheum Dis, 68, 1878, 10.1136/ard.2008.095836 · DOI
  20. Smedema, 2005, Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis, Am Coll Cardiol, 45, 1683, 10.1016/j.jacc.2005.01.047 · DOI
  21. Mahrholdt, 2005, Delayed enhancement cardiovascular magnetic resonance assessment of non-ischaemic cardiomyopathies, Eur Heart J, 26, 1461, 10.1093/eurheartj/ehi258 · DOI
  22. Kühl, 1996, Immunohistological evidence for a chronic intramyocardial inflammatory process in dilated cardiomyopathy, Heart, 75, 295, 10.1136/hrt.75.3.295 · DOI
  23. West, 1981, Association of myositis and myocarditis in progressive systemic sclerosis, Arthritis Rheum, 24, 662, 10.1002/art.1780240506 · DOI
  24. Clemson, 1992, Acute myocarditis in fulminant systemic sclerosis, Chest, 101, 872, 10.1378/chest.101.3.872 · DOI
  25. Carette, 1985, Severe myositis and myocarditis in progressive systemic sclerosis, J Rheumatol, 12, 997
  26. Kerr, 1993, Myocarditis as a complication in scleroderma patients with myositis, Clin Cardiol, 16, 895, 10.1002/clc.4960161212 · DOI
  27. Liangos, 2000, The possible role of myocardial biopsy in systemic sclerosis, Rheumatology, 39, 674, 10.1093/rheumatology/39.6.674 · DOI
  28. Stack, 2010, Successful control of scleroderma myocarditis using a combination of cyclophosphamide and methylprednisolone, Scand J Rheumatol, 39, 349, 10.3109/03009740903493741 · DOI
  29. Ferri, 1999, Parvovirus B19 infection of bone marrow in systemic sclerosis patients, Clin Exp Rheumatol, 17, 718
  30. Ohtsuka, 2004, Increased prevalence of human parvovirus B19 DNA in systemic sclerosis skin, Br J Dermatol, 150, 1091, 10.1111/j.0007-0963.2004.05930.x · DOI
  31. 1980, Preliminary criteria for the classification of systemic sclerosis (scleroderma), Arthritis Rheum, 23, 581, 10.1002/art.1780230510 · DOI
  32. De Cobelli, 2006, Delayed gadolinium-enhanced cardiac magnetic resonance in patients with chronic myocarditis presenting with heart failure or recurrent arrhythmias, J Am Coll Cardiol, 47, 1649, 10.1016/j.jacc.2005.11.067 · DOI
  33. De Santis, 2005, Functional, radiological and biological markers of alveolitis and infections of the lower respiratory tract in patients with systemic sclerosis, Respir Res, 6, 96, 10.1186/1465-9921-6-96 · DOI
  34. Pieroni, 2009, High prevalence of myocarditis mimicking arrhythmogenic right ventricular cardiomyopathy differential diagnosis by electroanatomic mapping-guided endomyocardial biopsy, J Am Coll Cardiol, 53, 681, 10.1016/j.jacc.2008.11.017 · DOI
  35. Angelini, 2002, Active versus borderline myocarditis: clinicopathological correlates and prognostic implications, Heart, 87, 210, 10.1136/heart.87.3.210 · DOI
  36. Aretz, 1987, Myocarditis: the Dallas Criteria, Hum Pathol, 18, 619, 10.1016/S0046-8177(87)80363-5 · DOI · Paper page
  37. Elamm, 2012, Pathogenesis and diagnosis of myocarditis, Heart, 98, 835, 10.1136/heartjnl-2012-301686 · DOI
  38. Galiè, 2009, Eur Heart J, 30, 2493, 10.1093/eurheartj/ehp297 · DOI
  39. Mavrogeni, 2009, Myocardial inflammation in autoimmune diseases: investigation by cardiovascular magnetic resonance and endomyocardial biopsy, Inflamm Allergy Drug Targets, 8, 390, 10.2174/1871528110908050390 · DOI
  40. Allanore, 2010, Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis, Ann Rheum Dis, 69, 218, 10.1136/ard.2008.103382 · DOI
  41. Ong, 2011, Usefulness of pericardial effusion as new diagnostic criterion for noninvasive detection of myocarditis, Am J Cardiol, 108, 445, 10.1016/j.amjcard.2011.03.068 · DOI
  42. Byers, 1997, Pericardial involvement in systemic sclerosis, Ann Rheum Dis, 56, 393, 10.1136/ard.56.6.393 · DOI
  43. Hashimoto, 2010, Microscopic polyangiitis in systemic sclerosis, Int J Rheumatol, 2010, 148528, 10.1155/2010/148528 · DOI
  44. Casari, 2002, Antineutrophil cytoplasmic antibodies a “red flag” in patients with systemic sclerosis, J Rheumatol, 29, 2666
  45. Khanna, 2003, Bactericidal/permeability-increasing protein and cathepsin G are the major antigenic targets of antineutrophil cytoplasmic autoantibodies in systemic sclerosis, J Rheumatol, 30, 1248
  46. Rho, 2006, Scleroderma associated with ANCA-associated vasculitis, Rheumatol Int, 26, 369, 10.1007/s00296-005-0011-5 · DOI
  47. Endo, 1994, Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis, J Rheumatol, 21, 864
  48. Rychlik-Golema, 2006, The role of endothelin-1 and selected cytokines in the pathogenesis of Raynaud's phenomenon associated with systemic connective tissue diseases, Int Angiol, 25, 221
  49. Kahaleh, 1999, Autoimmunity and vascular involvement in systemic sclerosis (SSc), Autoimmunity, 31, 195, 10.3109/08916939908994064 · DOI
  50. Varga, 2007, Systemic sclerosis: a prototypic multisystem fibrotic disorder, J Clin Invest, 117, 557, 10.1172/JCI31139 · DOI
  51. Yamashita, 2011, IL-6-mediated Th17 differentiation through RORγt is essential for the initiation of experimental autoimmune myocarditis, Cardiovasc Res, 91, 640, 10.1093/cvr/cvr148 · DOI
  52. Yang, 2005, The role of endothelin-1 in myocarditis and inflammatory cardiomyopathy: old lessons and new insights, Can J Physiol Pharmacol, 83, 47, 10.1139/y05-002 · DOI
  53. Pengo, 2012, What have we learned about antiphospholipid syndrome from patients and antiphospholipid carrier cohorts?, Semin Thromb Hemost, 38, 322, 10.1055/s-0032-1304719 · DOI
  54. Allanore, 2006, Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and Tissue-Doppler echography study, J Rheumatol, 33, 2464
  55. Kanno, 2011, Systemic sclerosis presented as congestive heart failure: an autopsy case, Mod Rheumatol, 21, 423, 10.3109/s10165-011-0413-z · DOI
  56. Neidhart, 1999, Increased serum levels of antibodies against human cytomegalovirus and prevalence of autoantibodies in systemic sclerosis, Arthritis Rheum, 42, 389, 10.1002/1529-0131(199902)42:2<389::AID-ANR23>3.0.CO;2-P · DOI
  57. Zakrzewska, 2009, Human Parvovirus B19 (B19V) infection in systemic sclerosis patients, Intervirology, 52, 279, 10.1159/000232945 · DOI
  58. Kühl, 2003, Parvovirus B19 infection mimicking acute myocardial infarction, Circulation, 108, 945, 10.1161/01.CIR.0000085168.02782.2C · DOI
  59. Bock, 2010, Human parvovirus B19-associated myocarditis, N Engl J Med, 362, 1248, 10.1056/NEJMc0911362 · DOI

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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
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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