Type:
Educational Exhibit
Keywords:
Tissue characterisation, Inflammation, Connective tissue disorders, Diagnostic procedure, MR, Cardiovascular system, Cardiac
Authors:
V. Bordonaro1, G. Rovere2, V. Vingiani2, R. Marano1, A. Meduri2, B. Merlino1, L. Natale2, R. Manfredi1; 1Rome/IT, 2Roma/IT
DOI:
10.26044/ecr2019/C-3426
Background
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.
Generally,
the clinical manifestations of ACTDs are characterized by a wide variability and the clinical management is mainly driven by symptoms due to articular,
cutaneous,
cardiopulmonary,
renal or neurological involvement.
The prevalence of cardiovascular involvement in ACTDs has long been underestimated: cardiac alterations often remain silent and largely undetected as they culminate in potentially severe conditions such as dilated cardiomyopathy (DCM),
rapidly evolving heart failure (HF) and arrhythmias.