Azam Ansari

Cardiovascular manifestations of systemic lupus erythematosus: Current perspective

Cardiovascular manifestations develop in the majority of SLE patients at some time during the course of their illness, the most common being acute fibrinous pericarditis and pericardial effusion. Echocardiography has demonstrated an increased incidence of pericardial effusion, even in those who have minimal symptoms. Chronic adhesive pericarditis, pericardial tamponade, and constrictive pericarditis occur rarely. While myocarditis is commonly noted at autopsy, it is often silent clinically. Diagnosis during life can be confirmed only by endomyocardial biopsy. Electrocardiographic changes are often nonspecific. Endocarditis with superimposed nonbacterial verrucous vegetations (Libman-Sacks) is noted in more than 40% of hearts at autopsy, but is rarely diagnosed during life. Valve dysfunctions, such as aortic stenosis, aortic insufficiency, mitral stenosis, and mitral insufficiency, occasionally manifest during life and rarely may necessitate surgery. Atrial and ventricular arrhythmias, first degree AV block, and acquired CHB occur in association with pericarditis, myocarditis, vasculitis, and myocardial fibrosis, respectively. CCHB developing in newborns of mothers with SLE, particularly those who have an antibody to soluble tissue ribonuclear protein RO(SS-A), is increasingly being appreciated by both pediatric cardiologists and rheumatologists. Recently, severe coronary atherosclerosis resulting in angina pectoris and/or myocardial infarction in young adults has been noted, particularly in those who had developed risk factors such as hypertension and hyperlipidemia while receiving prolonged corticosteroid therapy. Rarely, coronary arteritis may produce similar symptoms. Congestive heart failure of either single or multiple etiologies carries an ominous prognosis. It remains a cause of high morbidity and mortality unless recognized early and treated properly. Extracardiac vascular manifestations of SLE include telangiectasia, vasculitis, livedo reticularis, Raynauds phenomena, and thrombophlebitis, all of which may occur either alone or in different combinations. Evidence is now slowly accumulating that substantiates that immune complex deposition, complement activation and subsequent inflammatory reaction is responsible for the majority of the cardiovascular manifestations of SLE, for example, pericarditis, myocarditis, endocarditis, coronary arteritis, coronary atherosclerosis, and systemic and pulmonary vasculitis.(ABSTRACT TRUNCATED AT 400 WORDS)

Vascular Manifestations of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a well-known acute and/or chronic multisystem disease of complex autoimmune nature, having predilection for cardiovascular system. While its cardiac manifestations have been adequately studied, there is paucity of information on its vascular manifestations. Accord ingly, we studied the incidence of vascular manifestations in 50 consecutive SLE patients seen at our institutions and in private practice during the past 12 years. Systemic hypertension (44%) was the most common vascular manifestation followed by vasculitis (30%), Raynauds phenomenon (26%), telangiectasis (20%), premature coronary atherosclerosis (6%), digital ulceration (6%), thrombophlebitis (6%), pulmonary hypertension (4%) and portal hypertension (4%). Diffuse systemic vasculitis similar to polyarteritis nodosa was rare (2%). Often more than one lesion was found in the same patient. The clinical diagnosis of these vascular manifestations in the context of the primary disease (SLE) usually does not pose any difficulty except when they antedate it. We also studied the pathology and pathogenesis of some of these vascular lesions in both autopsy and biopsy specimens by both light microscopy and immunofluorescent techniques. Our results as well as those of others who also studied these lesions indicate that immune complex deposition and subsequent complement activation play an important role in the pathogenesis of vasculitis, coronary arteritis and premature coronary atherosclerosis. Corticosteroids and vasodilators remain the drugs of choice for the manage ment of the majority of the symptoms arising from the vascular lesions of SLE.

Syndromes of cardiac cachexia and the cachectic heart: Current perspective

T HE HUMAN HEART can be described as a chemodynamic machine that liberates energy stored in carbon-carbon and carbonhydrogen bonds of substrate fuels, such as carbohydrates, proteins, and fat, and uses this energy to perform the mechanical work positively and efficiently.’ In order to perform this task the human heart relies heavily on a reciprocal state of normal interdependence between its own nutritional state and that of the host. Any disturbance or aberration of this normal interdependence, such as hyperlipidemia, hypertension, alcohol abuse, and malnutrition, can result in disease states such as aortocoronary atherosclerosis, hypertensive cardiovascular disease, alcoholic cardiomyopathy, and nutritional cardiomyopathy. The purpose of this article is not to discuss the above-mentioned disease entities, regarding which an exhaustive amount of literature is already available. Instead, the intention is to discuss the diagnosis, recent developments in the pathogenesis, and management of cardiac cachexia and the cachectic heart. Normally, loss or gain of weight, body fat, and body mass results from an imbalance between energy intake and expenditure.2 However, for several decades it has been known that patients with chronic congestive cardiac failure (CCHF) with a prominent right-sided component may suffer from a symptom complex known as syndrome of cardiac cachexia (SOCC), evidenced by weight loss and wasting of body fat and lean body mass. Its precise pathogenesis and where the imbalance of energy intake and expenditure lies remain obscure, but the incidence and severity of resulting cachexia usually bears a direct relationship to the duration and the severity of underlying heart disease that is usually valvular in etiology. Similarly, patients with malignant epithelial and mesenchymal tumors, malignant lymphoma, far advanced pulmonary tuberculosis, advanced cirrhosis of liver, and severe malnutrition resulting from malabsorption of any cause also suffer severe weight loss and wasting of body fat and lean body mass due to calorie expenditure presumably exceeding the intake,3 and this pathologic process does not spare the cardiovascular system. The latter observation is contrary to the past notions of several noted physiologists who not only maintained but also preserved the long-standing myth that the human heart is resistant to chronic malnutrition and does not undergo atrophy and degeneration easily, as do other organs of the body.4 This earlier concept probably evolved from an erroneous belief. that “nature protects the most vital organs,” ie, the heart and brain, during chronic malnutrition. Though philosophically and theologically attractive, this concept is not supported by the clinicopathologic observations of the syndrome of the cachectic heart (SOCH). This entity, when first described in 1968 by Burch et al,’ was defined as an acquired pathologic decrease in the size and mass of the heart along with a decrease in the epicardial fat with little or no aortocoronary atherosclerosis. Certain clinical, ECG, roentgenographic, and pathologic correlations were required for the diagnosis to be made. Even though the terminology used to define SOCC and SOCH is sufficiently distinctive and descriptive, the possibility for confusion between them exists due to certain unavoidable overlapping terms. In order to avoid such confusion, the basic ingredients and concepts of these two different and distinct syndromes, which in author’s view represent two unique and fascinating experiments of nature, are schematically illustrated in Fig 1.