Heart involvement in autoimmune rheumatic diseases: the “phantom of the opera”

The mystery of the interplay between the heart and joints is as vivid today as it was in the ancient times. Whether it is the seat of the soul, the seat of passion and emotions, or simply the subject of poet’s ramblings, the heart is vital to human life and to human culture. Hippocrates (460–377 BC) was aware that despite the fact that it is only the size of a fist, it is still central to human existence. He was also the one who rejected the ruling views that illness is caused by possession of evil spirits and suggested ailments of the joints. He provided convincing descriptions of gout, rheumatoid arthritis, scleroderma, and Behcet’s disease and noticed diseases of the heart in the same patients 1. Nowadays, under dynamic pace of technological evolution and chronic stress, the heart is not any more the most ‘noble’ organ resistant to disease, but also the common victim in the universe of the human body. Therefore, the treatment of heart disease has a critical role in human medicine, and has been a driving strategy for the introduction of various attractive methodologies able to change the health perspectives of the mankind on the global scale. Since Hippocrates time, the early diagnosis of heart disorder remains the mandatory precaution to prevent myocardial damage and failure. Cardiovascular manifestations in autoimmune rheumatic diseases (ARD) have been infrequently recognized and mainly neglected in the medical community, despite the very clear evidence of the high death toll due to extra-articular involvement in patients affected by ARD. Therefore, as in the ‘Phantom of the Opera’ scenario, the main attention of rheumatologists has been focused on the playing ‘opera’—joints in rheumatoid arthritis (RA), skin, kidney, and lung in systemic sclerosis (SSc), kidney and brain in systemic lupus erythematosus (SLE). The heart has always been hidden behind the curtains, a ‘phantom’ that has silently killed when the rumour of the ‘orchestra’ of all other organs was for sure an attractive distraction to our eyes. In the last several decades, the increased prevalence of cardiovascular manifestation in patients with ARD was observed, which contributed substantially to their morbidity and mortality. Both comprehensive understanding of aetiopathogenesis leading to improved classification of ARD, and detailed and accurate cardiac imaging techniques allowed accurate diagnosis. Cardiac involvement may represent the initial manifestation of an ARD and cardiologists should remain vigilant to recognize the clinical pattern of involvement that may provide the clue to the underlying disease. Cardiac manifestations range from unusual to prominent and from mild to dramatic. Occult cardiovascular involvement is common, but frequently detectable by non-invasive methods. Although ARD affects all cardiac structures, pericarditis is the most common manifestation, while endocardial, myocardial, and coronary involvement occurs in distinct patterns (Fig. 1). Heart disease in ARD develops through several pathophysiological mechanisms including myocardial inflammation and/or fibrosis, infiltration by granulomatous tissue, vasculitis, thrombus formation, or accelerated coronary atherosclerosis. Beyond this, pulmonary arterial hypertension leads to significant cardiac dysfunction and rapid progression of heart failure.