title: Early myocardial and skeletal muscle interstitial remodelling in systemic sclerosis: insights from extracellular volume quantification using cardiovascular magnetic resonance.
abstract: OBJECTIVE
  Systemic sclerosis (SSc) may induce cardiac fibrosis and systo-diastolic dysfunction. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis with late gadolinium enhancement (LGE) and interstitial myocardial fibrosis with T1 mapping techniques. The aim of the study was to detect subclinical cardiac involvement with CMR in paucisymptomatic SSc patients with no previous history of myocardial disease, comparing it with skeletal muscle remodelling.
  
  
  RESULTS
  Thirty consecutive SSc patients (mean age: 51 ± 12 years, all women) and 10 healthy controls (mean age: 48 ± 15 years, all women) underwent clinical, biohumoral assessment, and CMR. Extracellular volume fraction (ECV) was calculated from pre- and post-contrast T1 values in the myocardium and skeletal muscle. Seventeen patients (57%) were asymptomatic, 13 (43%) paucisymptomatic (effort dyspnoea). All patients had normal biventricular volumes and systolic function, while LGE was present in seven patients (23%). Myocardial ECV was significantly increased in patients with SSc (30 ± 4%) than controls (28 ± 4%, P = 0.03), as was skeletal muscle ECV (23 ± 6% vs. 18 ± 4%, P < 0.01). Myocardial ECV did not differ between patients with and without LGE (P = NS) and showed no significant correlations with clinical data, biventricular volumes, systolic, or diastolic function. Overall, myocardial ECV showed a significant correlation with skeletal muscle ECV (R = 0.58, P < 0.001).
  
  
  CONCLUSIONS
  SSc is associated not only with myocardial replacement fibrosis, as detected by LGE, but also with interstitial remodelling of the myocardium and skeletal muscles, as detected by an increased ECV also in patients with normal biventricular function, with potential diagnostic, prognostic, and therapeutic clinical implications.
authors.affiliation: Fondazione Toscana 'Gabriele Monasterio', CNR, Regione Toscana, Pisa, Italy Institute of Life Sciences, Scuola Superiore 'Sant'Anna', Pisa, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Barison
authors.affiliation: Clinical Physiology Institute, National Research Council, CNR, Pisa, Italy.
authors.forename: Luna
authors.initials: L
authors.lastname: Gargani
authors.affiliation: Fondazione Toscana 'Gabriele Monasterio', CNR, Regione Toscana, Pisa, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: De Marchi
authors.affiliation: Fondazione Toscana 'Gabriele Monasterio', CNR, Regione Toscana, Pisa, Italy.
authors.forename: Giovanni Donato
authors.initials: GD
authors.lastname: Aquaro
authors.affiliation: Department of Rheumatology, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Guiducci
authors.affiliation: Clinical Physiology Institute, National Research Council, CNR, Pisa, Italy.
authors.forename: Eugenio
authors.initials: E
authors.lastname: Picano
authors.affiliation: Department of Rheumatology, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Italy.
authors.forename: Marco Matucci
authors.initials: MM
authors.lastname: Cerinic
authors.affiliation: Clinical Physiology Institute, National Research Council, CNR, Pisa, Italy pingi@ifc.cnr.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Pingitore
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D015331:Cohort Studies; D004719:Endomyocardial Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D005682:Gadolinium; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000209:etiology / Q000473:pathology*; D011446:Prospective Studies; D011856:Radiographic Image Enhancement; D012016:Reference Values; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D018709:Statistics, Nonparametric; D013997:Time Factors; D016277:Ventricular Function, Left / Q000502:physiology; D016278:Ventricular Function, Right / Q000502:physiology; D020257:Ventricular Remodeling / Q000502:physiology*
chemical_list: D005682:Gadolinium
keywords: Cardiac T1 mapping; Cardiovascular magnetic resonance; Late gadolinium enhancement; Myocardial fibrosis; Systemic sclerosis