Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: Potential utility of immunosuppressive therapy in cardiac damage progression

Maurizio Pieroni, Maria De Santis, Gaetano Zizzo, Silvia Bosello, Costantino Smaldone, Mara Campioni, Giacomo De Luca, Antonella Laria, Agostino Meduri, Fulvio Bellocci, Lorenzo Bonomo, Filippo Crea, Gianfranco Ferraccioli

Research output: Contribution to journalArticle

58 Citations (Scopus)

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. Immunosuppressive therapy improved symptoms and led to cardiac enzyme negativization; however, 2 patients died of sudden death during follow-up. Conclusions: Myocarditis is a common finding in SSc patients with recent-onset cardiac involvement. Its early detection allowed to timely start an immunosuppressive treatment, preventing cardiac damage progression in most cases.

Original languageEnglish
Pages (from-to)526-535
Number of pages10
JournalSeminars in Arthritis and Rheumatism
Volume43
Issue number4
DOIs
Publication statusPublished - Feb 2014

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Systemic Scleroderma
Myocarditis
Immunosuppressive Agents
Heart Diseases
Therapeutics
Ventricular Premature Complexes
Pericardial Effusion
Antineutrophil Cytoplasmic Antibodies
Parvovirus
Myositis
Magnetic Resonance Angiography
Enzymes
Sudden Death
Microvessels
Coronary Angiography
Chest Pain
Radionuclide Imaging
Muscle Cells
Endothelium
Echocardiography

Keywords

  • ANCA
  • Heart disease
  • Myocarditis
  • Parvovirus B19
  • Systemic sclerosis
  • Treatment

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine

Cite this

Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease : Potential utility of immunosuppressive therapy in cardiac damage progression. / Pieroni, Maurizio; De Santis, Maria; Zizzo, Gaetano; Bosello, Silvia; Smaldone, Costantino; Campioni, Mara; De Luca, Giacomo; Laria, Antonella; Meduri, Agostino; Bellocci, Fulvio; Bonomo, Lorenzo; Crea, Filippo; Ferraccioli, Gianfranco.

In: Seminars in Arthritis and Rheumatism, Vol. 43, No. 4, 02.2014, p. 526-535.

Research output: Contribution to journalArticle

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 2014, 'Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: Potential utility of immunosuppressive therapy in cardiac damage progression', Seminars in Arthritis and Rheumatism, vol. 43, no. 4, pp. 526-535. https://doi.org/10.1016/j.semarthrit.2013.07.006
Pieroni, Maurizio ; De Santis, Maria ; Zizzo, Gaetano ; Bosello, Silvia ; Smaldone, Costantino ; Campioni, Mara ; De Luca, Giacomo ; Laria, Antonella ; Meduri, Agostino ; Bellocci, Fulvio ; Bonomo, Lorenzo ; Crea, Filippo ; Ferraccioli, Gianfranco. / Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease : Potential utility of immunosuppressive therapy in cardiac damage progression. In: Seminars in Arthritis and Rheumatism. 2014 ; Vol. 43, No. 4. pp. 526-535.
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AU - Pieroni, Maurizio

AU - De Santis, Maria

AU - Zizzo, Gaetano

AU - Bosello, Silvia

AU - Smaldone, Costantino

AU - Campioni, Mara

AU - De Luca, Giacomo

AU - Laria, Antonella

AU - Meduri, Agostino

AU - Bellocci, Fulvio

AU - Bonomo, Lorenzo

AU - Crea, Filippo

AU - Ferraccioli, Gianfranco

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N2 - 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. Immunosuppressive therapy improved symptoms and led to cardiac enzyme negativization; however, 2 patients died of sudden death during follow-up. Conclusions: Myocarditis is a common finding in SSc patients with recent-onset cardiac involvement. Its early detection allowed to timely start an immunosuppressive treatment, preventing cardiac damage progression in most cases.

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KW - Myocarditis

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KW - Systemic sclerosis

KW - Treatment

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