Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: potential utility of immunosuppressive therapy in cardiac damage progression.
OBJECTIVE
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.
Division of Cardiology, Catholic University of the Sacred Heart, Rome, Italy.
Maurizio
M
Pieroni
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Maria
M
De Santis
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Gaetano
G
Zizzo
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Silvia
S
Bosello
Division of Cardiology, Catholic University of the Sacred Heart, Rome, Italy.
Costantino
C
Smaldone
Division of Cardiology, Catholic University of the Sacred Heart, Rome, Italy.
Mara
M
Campioni
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Giacomo
G
De Luca
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Antonella
A
Laria
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy.
Agostino
A
Meduri
Division of Cardiology, Catholic University of the Sacred Heart, Rome, Italy.
Fulvio
F
Bellocci
Institute of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
Lorenzo
L
Bonomo
Division of Cardiology, Catholic University of the Sacred Heart, Rome, Italy.
Filippo
F
Crea
Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, CIC-Via Moscati 31, Rome 00168, Italy. Electronic address: gf.ferraccioli@rm.unicatt.it.
Gianfranco
G
Ferraccioli
D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
D007166:Immunosuppressive Agents
ANCA; Heart disease; Myocarditis; Parvovirus B19; Systemic sclerosis; Treatment