Targeted therapies in connective tissue diseases (CTDs) have led to improvements of disease-associated outcomes, but life expectancy remains lower compared to general population due to emerging co-morbidities, particularly due to excess cardiovascular risk. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging technique which can provide detailed information about multiple cardiovascular pathologies without using ionizing radiation. CMR is considered the reference standard for quantitative evaluation of left and right ventricular volumes, mass and function, cardiac tissue characterization and assessment of thoracic vessels; it may also be used for the quantitative assessment of myocardial blood flow with high spatial resolution and for the evaluation of the proximal coronary arteries. These applications are of particular interest in CTDs, because of the potential of serious and variable involvement of the cardiovascular system during their course. The International Consensus Group on CMR in Rheumatology was formed in January 2012 aiming to achieve consensus among CMR and rheumatology experts in developing initial recommendations on the current state-of-the-art use of CMR in CTDs. The present report outlines the recommendations of the participating CMR and rheumatology experts with regards to: (a) indications for use of CMR in rheumatoid arthritis, the spondyloarthropathies, systemic lupus erythematosus, vasculitis of small, medium and large vessels, myositis, sarcoidosis (SRC), and scleroderma (SSc); (b) CMR protocols, terminology for reporting CMR and diagnostic CMR criteria for assessment and quantification of cardiovascular involvement in CTDs; and (c) a research agenda for the further development of this evolving field.

Objective. Accurate diagnosis of cardiovascular involvement in connective tissue diseases (CTDs) remains challenging. We hypothesized that cardiovascular magnetic resonance (CMR) demonstrates cardiac lesions in symptomatic CTD patients with normal echocardiography. Methods. CMR from 246 CTD patients with typical cardiac symptoms (TCS; n ‫؍‬ 146, group A) or atypical cardiac symptoms (ATCS; n ‫؍‬ 100, group B) was retrospectively evaluated. Group A included 9 patients with inflammatory myopathy (IM), 35 with sarcoidosis, 30 with systemic sclerosis (SSc), 14 with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA), and 48 with small vessel vasculitis. Group B included 25 patients with RA, 20 with SLE, 20 with sarcoidosis, 15 with SSc, 10 with IM, and 10 with small vessel vasculitis. CMR was performed by 1.5T; left ventricular ejection fraction, T2 ratio (edema imaging), and late gadolinium enhancement (LGE; fibrosis imaging) were evaluated. Acute and chronic lesions were characterized as LGE positive plus T2 ratio >2 and T2 ratio <2, respectively. According to LGE, lesions were characterized as diffuse subendocardial, subepicardial, and subendocardial/transmural due to vasculitis, myocarditis, and myocardial infarction, respectively. A stress study by dobutamine echocardiography or stress, nuclear, or adenosine CMR was performed in CTD patients with negative rest CMR. Results. Abnormal CMR was identified in 32% (27% chronic) and 15% (12% chronic) of patients with TCS and ATCS, respectively. Lesions due to vasculitis, myocarditis, and myocardial infarction were evident in 27.4%, 62.6%, and 9.6% of CTD patients, respectively. Stress studies in CTD patients with negative CMR revealed coronary artery disease in 20%. Conclusion. CMR in symptomatic CTD patients with normal echocardiography can assess disease acuity and identify vasculitis, myocarditis, and myocardial infarction.

CMR may reveal severe cardiac involvement in early, asymptomatic diffuse SSc with normal routine cardiac evaluation, presenting either as myocardial inflammation or as severe reduction of MPRI and diffuse fibrosis with further deterioration in the long term follow up.

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Objectives Cardiac rhythm disturbances constitute the most frequent cardiovascular cause of death in SSc. However, electrocardiographic findings are not a part of risk stratification in SSc. We aimed to translate 24 h Holter findings into a tangible risk prediction score using cardiovascular magnetic resonance. Methods The Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS) was a prospective multicentre study including 150 consecutive SSc patients from eight European centres, assessed with 24 h Holter and cardiovascular magnetic resonance, including ventricular function, oedema (T2 ratio) and late gadolinium enhancement (%LGE). Laboratory/clinical parameters were included in multivariable corrections. A combined endpoint of sustained ventricular tachycardia requiring hospitalization and sudden cardiac death at a median (interquartile range) follow-up of 1 (1.0–1.4) year was generated. Results Only T2 ratio and %LGE were significant predictors of ventricular rhythm disturbances, but not of supraventricular rhythm disturbances, after multivariable correction and adjustment for multiple comparisons. Using decision-tree analysis, we created the SAnCtUS score, a four-category scoring system based on T2 ratio and %LGE, for identifying SSc patients at high risk of experiencing ventricular rhythm disturbance at baseline. Increasing SAnCtUS scores were associated with a greater disease and arrhythmic burden. All cases of non-sustained ventricular tachycardia (n = 7) occurred in patients with the highest SAnCtUS score (=4). Having a score of 4 conveyed a higher risk of reaching the combined endpoint in multivariable Cox regression compared with scores 1/2/3 [hazard ratio (95% CI): 3.86 (1.14, 13.04), P = 0.029] independently of left ventricular ejection fraction and baseline ventricular tachycardia occurrence. Conclusion T2 ratio and %LGE had the greatest utility as independent predictors of rhythm disturbances in SSc patients.