To analyze the role of CMR in the evaluation of myocardial injury in Patients (Pts) with Autoimmune Connective Tissue Disorders (ACTD)
To investigate the different pathophysiological mechanisms underlying myocardial damage in the various forms of ACTD and their correlation with main patterns of presentation at imaging.
ACTDs are a heterogeneous group of autoimmune diseases with systemic involvement,
characterized by progressively irreversible multi-organ damage.
The most common forms of ACTDs include scleroderma (systemic sclerosis - SSc),
systemic lupus erythematosus (SLE),
inflammatory myopathies and Sjögren's syndrome.
Mixed connective tissue diseases (mixed-ACTDs) should be considered in Pts with overlapping clinical features,
in which there is an autoimmune condition that does not meet the criteria for a specific form.
the clinical manifestations of ACTDs are characterized by a wide variability and the clinical management...
Findings and procedure details
Pathophysiology of cardiac involvement in ACTDs
Cardiac abnormalities in ACTDs are the consequence of multiple pathophysiological mechanisms which lead to a progressive development of a myocardial fibrosis (Table 1):
- presence of a chronic systemic inflammation;
- accelerated atherosclerosis with abnormal reactivity of the vessels against systemic inflammation;
- myocardial ischemia injury caused by alteration of both micro- and macrovascular circulation;
- cardiotoxicity of anti-rheumatic drugs.
Early pathophysiological researches postulated the crucial role of accelerated vascular injury,
in which the co-existence of endothelial alterations,
The timely recognition of myocardial involvement in ACTDs is crucial in their clinical management,
in order to prevent major complications and improve the prognosis.
CMR allows an accurate assessment of myocardial injury,
even in a subclinical phase,
and it is useful for risk stratification and follow-up,
guiding the optimization of immunosuppressive and cardiovascular therapy.
Deciphering cardiac involvement in systemic inflammatory diseases: noninvasive tissue characterisation using cardiac magnetic resonance is key to improved patients’ care.Expert review of cardiovascular therapy,14(11),
Cardiovascular magnetic resonance in rheumatology: current status and recommendations for use.International journal of cardiology,217,
Imaging of systemic lupus erythematosus.