Systemic inflammatory diseases are associated with an increase in cardiovascular (CV) morbidity and mortality. CV involvement in systemic inflammatory diseases is highly heterogeneous, with manifestations affecting different heart structures. New instruments now allow an early diagnosis and prompt treatment. Over the last few years, Cardiac MRI has emerged as a noninvasive means of accurately and reproducibly assessing myocardial anatomy and function and has become the gold standard for measuring the ejection fraction, and left and right ventricular volumes. It can reveal myocardial lesions in patients with mixed connective tissue disease (CTD) and cardiac symptoms, including myocardial infarction, inflammation, and widespread subendocardial fibrosis and perfusion defects, which require further cardiac investigation and/or treatment. Computed tomography (CT) has evolved into such a potent means of diagnosis. It has become very important for cardiologists because it is widely available, easy to use, noninvasive, and highly sensitive in detecting coronary artery disease, and evaluating pericardial disease, pericardial fluid, and pericardial neoplasms. Radiation doses are a matter of concern and special measures need to be taken to avoid unnecessarily high doses when CT is used for coronary artery imaging. Pulmonary arterial hypertension (PAH) is one of the major complications of CTDs such as systemic sclerosis, systemic lupus erythematosus, and mixed CTD. Right heart catheterization is a fundamental means of confirming a diagnosis of PAH and assessing a risk that, when performed at dedicated centers, is associated with low morbidity and mortality rates. Myocarditis is an inflammatory disease of the myocardium caused by various infectious and noninfectious triggers. Systemic autoimmune diseases such as Churg–Strauss syndrome or hypereosinophilic syndrome (Loeffler's disease) can be associated with eosinophilic myocarditis. In the case of cardiac sarcoidosis and giant cell myocarditis, which are rare causes of inflammatory myocardial disease, early diagnosis and treatment significantly improve prognosis. The diagnostic gold standard is still an endomyocardial biopsy.