Heart involvement in autoimmune rheumatic diseases: the "phantom of the opera"
1Department of Medicine and Surgery, Division of Medicine I and Rheumatology University of Florence, AOUC, Florence, Italy and 1Department of Cardiology, Institute for Cardiovascular Diseases of the Clinical Centre of Serbia and Belgrade University Medical School, Belgrade, Serbia.
Correspondence to: M. Matucci Cerinic, Dip Medicina Interna, SOD Medicina Interna I e Reumatologia, Villa Monna Tessa, Viale Pieraccini 18, 50139 Firenze, Italia. E-mail: cerinic{at}unifi.it
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.
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The level of awareness the rheumatological community has reached regarding the evaluation and management of cardiovascular risk in RA is testified by the development of new clinical practice guidelines [2]. These guidelines recognized that RA is an independent cardiovascular risk factor and that persistent inflammation imposes an additional threat. The need for vigorous reduction of all modifiable risk factors and treatment with minimal dose of glucocorticoids was highlighted. Important for clinical practice is the positive effect of methotrexate therapy on the reduction of cardiovascular mortality and the contraindication for TNF-
antagonists in patients with severe heart failure. The importance of cardiovascular involvement in RA and its role in reducing the life expectancy have been elucidated in this issue of Rheumatology by Voskuyl [3]. There are few diseases attended by such a complex clinical presentation and difficult management as SSc. In SSc, in spite of the predominance of vascular abnormalities and documented ischemia, the incidence of coronary artery disease is not different in comparison to general population [4]. It is still not known whether coronary artery vasospasm or fibrosis of the arterioles, is the main factor leading to myocardial ischemia. However, sudden death due to fatal arrhythmia [5] and ventricular dilation, leading to congestive heart failure [6], demonstrates that the myocardium is a preferred target of the disease and remains a major threat to SSc patients. Detailed review on primary myocardial involvement in SSc is elegantly presented in the paper by Kahan and Allanore [7].
Autoantibodies can damage all heart structures (Fig. 1), not only in SLE patients but also in antiphospholipid syndrome (APS) and in the foetus of mothers affected by Sjögren syndrome. Autoantibodies may interact with target tissue or induce downstream mechanisms leading to cardiac lesions. Tincani and colleagues [8] comprehensively analysed this aspect of involvement of the heart in SLE, APS, and neonatal lupus.
In dermatomyositis and polymyositis, heart involvement may have fatal consequences, despite clinically overt involvement of the heart is seldom evident. In fact, subclinical manifestations, characterized by conduction abnormalities and arrhythmias, are frequently observed. This issue of Rheumatology is enriched by Lundberg's comprehensive analysis of heart involvement in dermatomyositis and polymyositis [9].
As nicely reviewed by Cutulo et al. [10], nailfold videocapillaroscopy is not only a useful diagnostic method for early diagnosis of SSc and poly/dermatomyositis, but also a feasible clinical tool to assess the microcirculation, which potentially correlates with the changes in the pulmonary vasculature [11]. Skin capillary density is significantly lower in patients with a reduced coronary flow reserve, due to the functional and structural alterations of the coronary microcirculation [12] and, in SSc, is often associated with both heart and renal involvement [13].
Pulmonary arterial hypertension is a major issue in ARD, especially SSc significantly affecting quality of life and prognosis of the patients. Distler and Pignone have made a valuable contribution to this volume by a state-of the-art analysis of diagnosis and treatment of pulmonary hypertension [14].
Major advances in the imaging techniques in cardiology significantly improved the understanding of pathophysiology and diagnostic yield in ARD. Diagnostic value of technological improvements in echocardiography, single photon emission computed tomography (SPECT), radionuclide ventriculography, angiography, computed tomography, and magnetic resonance imaging is evaluated in this issue of Rheumatology by Maksimovic et al. [15].
Rhythm and conduction disorders are not only a frequent clinical manifestation of cardiovascular involvement in ARD but also a possible cause of sudden death. Importance of early diagnosis and proper management of cardiac arrhythmias and conduction disturbances is elucidated in the contribution of Seferovic et al. to this issue [16].
As pointed out by Maisch and colleagues [17] invasive assessment including cardiac catheterisation, coronary angiography, and endomyocardial biopsy can distinguish between ischemic, inflammatory, or fibrotic myocardial lesions in ARD. Importantly, advances in interventional cardiology provide a new perspective for the management of coronary artery disease in ARD patients.
Basic research in imaging and rheumatology might provide a better insight into ARD pathogenesis and might guide development of targeted disease-specific therapies, while clinical research, both in rheumatology and cardiology, may provide optimized therapeutic solutions. Further progress in the treatment of the cardiovascular manifestations of ARD requires continuous diligence, timely strategic planning and cooperation between rheumatologists and cardiologists from the very early onset of ARD. Multicenter randomized controlled trials are required to draw firm conclusions for better insight in the detection and treatment of cardiovascular involvement in ARD. Keeping in mind the ‘Phantom of the Opera’ scenario, the alliance of rheumatologists and cardiologists is needed not only to be aware of the menace behind the curtains, but also to help each other to strategically prevent and fight the cardiovascular involvement in ARD, thus preserving the quality of life of our patients.
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