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.

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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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Background Cardiovascular disease (CVD) has been documented in >50% of systemic lupus erythematosus (SLE) patients, due to a complex interplay between traditional risk factors and SLE-related factors. Various processes, such as coronary artery disease, myocarditis, dilated cardiomyopathy, vasculitis, valvular heart disease, pulmonary hypertension and heart failure, account for CVD complications in SLE. Methods Electrocardiogram (ECG), echocardiography (echo), nuclear techniques, cardiac computed tomography (CT), cardiovascular magnetic resonance (CMR) and cardiac catheterization (CCa) can detect CVD in SLE at an early stage. ECG and echo are the cornerstones of CVD evaluation in SLE. The routine use of cardiac CT and nuclear techniques is limited by radiation exposure and use of iodinated contrast agents. Additionally, nuclear techniques are also limited by low spatial resolution that does not allow detection of sub-endocardial and sub-epicardial lesions. CCa gives definitive information about coronary artery anatomy and pulmonary artery pressure and offers the possibility of interventional therapy. However, it carries the risk of invasive instrumentation. Recently, CMR was proved of great value in the evaluation of cardiac function and the detection of myocardial inflammation, stress-rest perfusion defects and fibrosis. Results An algorithm for CVD evaluation in SLE includes clinical, laboratory, ECG and echo assessment as well as CMR evaluation in patients with inconclusive findings, persistent cardiac symptoms despite normal standard evaluation, new onset of life-threatening arrhythmia/heart failure and/or as a tool to select SLE patients for CCa. Conclusions A non-invasive approach including clinical, laboratory and imaging evaluation is key for early CVD detection in SLE.

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.

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Inflammation, microvascular and macrovascular ischemia, valvular disease, and fibrosis are the main causes of cardiovascular disease (CVD) in autoimmune rheumatic diseases (ARDs). The silent presentation and the high mortality and/or morbidity of CVD in ARDs necessitate a reliable tool for early diagnosis. Noninvasive cardiovascular imaging, including echocardiography, nuclear imaging, cardiovascular computed tomography (CT), cardiac magnetic resonance (CMR), and hybrid imaging modalities, constitutes the main tool for monitoring of CVD in ARDs. Echocardiography is the cornerstone for CVD evaluation, but it is operator-dependent and cannot perform tissue characterization. Nuclear imaging and CT, although promising, have the disadvantage of ionizing radiation. CMR can assess inflammation, ischemia, and fibrosis without ionizing radiation, thus making it a necessary adjunct, which is especially relevant for ARDs with new-onset heart failure, conflicting data from other imaging modalities, and recent onset of chest pain and/or arrhythmias. Recently, hybrid imaging with positron emission tomography (PET)/CT and PET/CMR has shown promise in ARDs, although these modalities currently have prohibitive costs.