The cardiovascular manifestations of rheumatic diseases

PURPOSE OF REVIEW: To review the cardiovascular manifestations of several more common rheumatic conditions in the light of the recent reported literature. RECENT FINDINGS: Evidence that chronic inflammation is associated with the occurrence of cardiac events in people both with and without chronic inflammatory joint disease is emerging. Both atherosclerosis and rheumatic diseases, however, have a complicated cause, and it is likely that inflammation contributes to other environmental and host risk factors in these patients. Treatments used to suppress inflammation in many rheumatic conditions have the potential to reduce cardiovascular disease morbidity as well as improve musculoskeletal function. SUMMARY: Cardiovascular morbidity and mortality have been found to be increased in association with many of the rheumatic diseases. In particular, coronary heart disease seems to be associated with inflammatory rheumatic conditions. Whilst it is likely that chronic systemic inflammation promotes accelerated atherosclerosis in these patients, the mechanisms by which this occurs are complex and the effects of treatment and other cardiovascular risk factors need to be considered.     

内分泌疾病的神经肌肉表现

内分泌疾病的临床表现错综复杂,一些非特异性的病变如神经肌肉改变易于漏诊、误诊或误治.糖皮质激素分泌异常、甲状腺疾病、垂体功能紊乱、钙和维生素D代谢异常等多种内分泌代谢性疾病,均可以导致神经肌肉病变而呈现相应的临床特征,如肌肉软弱、肌肉萎缩、乏力等.通过对症处理以及对原发疾病的及时诊断和治疗,神经肌肉病变可以恢复或者缓解.     

Neuromuscular manifestations of electrolyte disorders

A large variety of diseases encountered in clinical medicine are associated with symptoms suggestive of neuromuscular dysfunction. The myalgia of viral prodromes, the muscular cramps of hyponatremia and the weakness of potassium deficiency are well known examples. Although investigation of the myopathic components of these illnesses is fragmentary, those that have been examined show abnormalities compatible with subtle alterations of ion transport and, when sought out, histologic changes of muscle fiber injury or necrosis. Certain electrolyte and divalent ion deficiencies are likely to cause such findings, especially those related to potassium and phosphorus. The precise mechanism by which cellular injury occurs in electrolyte derangements has not been clearly elucidated. However, in potassium deficiency, for example, at least three potentially harmful effects occur: (1) abnormally low muscle blood flow with exercise; (2) suppression of glycogen synthesis and storage in muscle; (3) deranged ion transport. In consonance with these observations, it can be shown that once a muscle cell is injured, even when the injury is subclinical, superimposition of any stress that demands substantial expenditure of energy so as to deplete muscle energy stores can apparently precipitate frank rhabdomyolysis. The combined influence of exericise and fasting is a prime example. New information based upon observations in patients and limited experimental studies in animals suggests that skeletal muscle cells, under conditions of certain specific electrolyte derangement, show a common pattern of changes characterized by abnormal electrogenesis (abnormal resting transmembrane potential difference) and elevation of intracellular Na concentration. Such changes could well set the stage for irreversible cellular injury by another important mechanism. Thus, depression of the normally high concentration ratio of sodium ions between extracellular and intracellular fluids or depression of membrane potential could impair the normal exchange of extracellular sodium for intracellular calcium ions. Impressive evidence suggests that if calcium ions thereby attain a critical elevated value in the sarcoplasm, autodestructive proteases are activated that can destroy the cell. The field of interest concerning electrolyte and divalent ion disorders and their implication in rhabdomyolysis is newly emerging. In this paper, the current information on these disorders is reviewed. With license, considerable speculation will be inserted on those disorders not yet adequately examined.     

Discordant spectrum of cardiac manifestations of neonatal lupus in twins

Congenital complete heart block associated with transplacental passage of maternal autoantibodies reactive with SSA/Ro and SSB/La is a rare disease with significant fetal, neonatal, and childhood morbidity and mortality. We present the case of dichorionic, diamniotic twins (female twin A and male twin B) exposed to maternal Ro and La autoantibodies with different disease expression. Twin A (female) had Mobitz type I second degree atrioventricular (AV) block (Wenckebach); twin B (male) had normal sinus rhythm. Both twins had structurally normal hearts but demonstrated echocardiographic evidence of endocardial fibroelastosis (EFE). Following maternal dexamethasone 4 mg once daily, twin A reverted to sinus rhythm in utero; twin B remained in sinus rhythm throughout pregnancy. Echocardiograms after delivery demonstrated resolution of EFE in both fetuses, and EKGs confirmed sinus rhythm. However, at five months of age, Holter monitor demonstrated first degree AV block and intermittent Wenckebach in twin A. Twin B remains in sinus rhythm. This case is one of only three in the literature that describes Mobitz type I second degree atrioventricular block presenting in fetuses exposed to maternal SSA and SSB autoantibodies and is the first case that we have seen reported in twins. Importantly, this case also adds to the growing body of literature describing EFE as a presentation of neonatal lupus with or without conduction system abnormalities, emphasizes the spectrum of cardiac conduction abnormalities in neonatal lupus syndrome, and raises interesting questions about discordant disease expression in twins.     

Chemokines and their role in cardiovascular diseases

Leukocyte infiltration into inflammatory or injured tissues requires a variety of cell-associated and soluble factors that mediate the communications between circulating leukocytes and vascular cells. During the past decade, a superfamily of polypeptide leukocyte chemoattractants known as chemokines has been identified and demonstrated to selectively induce rapid endothelial cell adhesion and transmigration of leukocyte subpopulations. Chemokines are produced by virtually every mammalian somatic cell type in response to inflammatory and immunologic stimuli and have been detected in tissues of numerous disease states characterized by infiltration of distinct leukocyte subsets. Chemokines bind and activate cell surface receptors that belong to the seven transmembrane, G protein-coupled receptor superfamily. Several chemokine receptors have been identified as fusion cofactors for human immunodeficiency virus type 1 (HIV-1). Chemokines have also been shown to play a critical role in atherosclerosis and ischemic reperfusion injury. As chemokine research is a rapidly expanding area, it is the purpose of this brief review to summarize recent progress in the field and to focus on the involvement of chemokines in cardiovascular diseases.     

The expanding spectrum of systemic autoinflammatory disorders and their rheumatic manifestations

The authors review the genes, and their respective proteins, responsible for eight autoinflammatory conditions. Familial Mediterranean fever is caused by mutations in pyrin, which is the prototype of a new family of proteins belonging to the death-domain superfamily. This new group of proteins, which regulate apoptosis, inflammation, and cytokine processing, share an approximately 90-amino-acid N-terminal sequence called the PYRIN domain. Mutations in another PYRIN domain protein, termed cryopyrin, are responsible for three clinically defined illnesses, Muckle-Wells syndrome, familial cold autoinflammatory syndrome, and NOMID/CINCA. A related protein encoded by the gene is responsible for the Mendelian disorder, Blau syndrome, and also predisposes to Crohn disease. The gene responsible for PAPA syndrome has recently been identified as, and preliminary results from the authors' laboratory also implicate its protein product in these pathways. Lastly, the authors discuss the broadening genetic and clinical spectrum of TRAPS, an autoinflammatory syndrome resulting from mutations in the 55-kDa receptor for tumor necrosis factor.     

Role of cardiovascular imaging in systemic autoimmune diseases

Systemic autoimmune diseases are characterized by an excess of cardiovascular (CV) morbidity and mortality compared to the general population, mainly due to chronic inflammation that promotes the development of endothelial dysfunction and enhanced atherosclerosis. Early diagnosis of silent CV involvement is mandatory to improve the long term prognosis of these patients and CV imaging provides valuable information as a reliable diagnostic tool. Transthoracic echocardiography, with several applications (e.g. coronary flow reserve evaluation, tissue Doppler imaging, speckle tracking and the transesophageal approach), represents a first line evaluation, in association with biomarkers of endothelial dysfunction, such as asymmetric dimethylarginine. Nuclear medicine provides useful information on myocardial perfusion. The aim of this editorial is to provide a brief but complete review of the diagnostic tools available for screening and follow up of CV involvement in systemic autoimmune diseases.     

重视磁共振成像在心血管疾病诊断中的应用

近年来,随着软硬件技术的发展和完善,心血管磁共振成像（Mm）已经在临床诊断中发挥越来越重要的作用。MRI的无辐射、大视野、任意层面扫描以及高度的软组织分辨率综合了众多无创性检查的优点,其“一站式”（one-stop shop）检查特点可同时完成心脏的解剖结构、功能、心肌缺血和心肌活性的评估,对大血管和周围血管病的诊断可达到类似X线数字减影血管造影（DSA）的效果,而且,它还能够在分子影像学领域发挥其潜在价值。     

Assessment of pericardial diseases and cardiac masses with cardiovascular magnetic resonance

Imaging plays an important diagnostic and prognostic role in the assessment of pericardial diseases and cardiac tumors and in differentiating these conditions from other cardiac and noncardiac diseases. A number of imaging modalities are available for this task; each has advantages and limitation. Cardiovascular magnetic resonance (CMR) is a highly versatile imaging modality that provides detailed anatomical information, tissue characterization, cardiac function assessment, and evaluation of the impact of these conditions on hemodynamics. In this review we focus on the current state-of-the-art application of cardiovascular magnetic resonance in assessing pericardial diseases and cardiac tumors.     

An eye on sexually transmitted diseases: sexually transmitted diseases and their ocular manifestations

Ocular examination should be a part of the routine assessment of the patients seen at sexually transmitted diseases (STD) clinics due to the importance of these organs in the general wellbeing of patients. It is essential to keep an open eye on ocular signs and symptoms of patients with a history of exposure to common STD pathogens, to ensure prompt investigation and management of ocular complications of the STDs, which, if left unnoticed, otherwise could subject the patients to a great deal of anxiety and distress.     

The role of serotonin in cardiovascular diseases and their treatment

Summary Serotonin (5HT), discovered in the 1950s, has been the subject of renewed interest for several years, in particular due to the subdivision of 5HT receptors into various types. Concomitantly, several more or less selective agonists and antagonists for these various receptor subtypes have been developed. Although the physiologic relevance of 5HT remains largely unknown, its role in certain pathologic processes is widely accepted. Certain symptoms of the carcinoid syndrome, thromboembolic processes at the level of the microcirculation, and possibly also coronary spasm and peripheral vascular disease are likely to be associated, at least in part, with endogenous 5HT and serotonergic mechanisms. However, a primary and causative role for such mechanisms in essential hypertension seems unlikely. The blockade of peripheral 5HT₂ receptors with drugs may offer advantages, in particular in those disorders where an interaction between predamaged blood vessels and platelets is involved. Such a therapeutic approach seems to be a more generally applicable principle than the lowering of blood pressure as such, which appears not to be a general phenomenon provoked by 5HT₂-receptor blockade.     

Physiological cardiovascular adaptation in pregnancy--its significance for cardiac diseases

Stroke volume and heart rate rise at the very beginning of a pregnancy and decline after birth, over the course of months. Arterial blood pressure is lowered, plasma volume is increased while central venous pressure stays constant during pregnancy. The rise in cardiac output in early pregnancy can be induced quantitatively by oestrogen. The pregnancy-induced rise in cardiac output is based on the fairly general remodelling of the cardiovascular system. In a process of development, many portions of the cardiovascular system undergo programmed dilation (expansion): There is a programmed dilation of the heart, of the aorta, of the resistance vessels of the kidney and the resistance vessels of the placenta, and a programmed dilation of the venous system. All the changes favour the perfusion of the pregnant body. Cardiac dilation increases directly stroke volume, aortic dilation increases the susceptance (Windkessel function) of the aorta, the peripheral dilation increases the vascular conductance, and the venous dilation raises blood volume. Since the vascular conductance increase by peripheral dilation is higher than the increase in stroke volume, arterial pressure drops and evokes, via the baroreceptor reflex, an increase in heart rate; the increase in cardiac output occurs to an equal extent by an increase in stroke volume and an increase in heart rate. Compression of the caval vein by the pregnant uterus increases peripheral venous pressure and possibly slows down blood flow in the limbs. Increase in cardiac output means a burden for the heart, especially when associated with increase in heart rate. In this condition, cardiac energy expenditure is increased while oxygen supply is decreased. The rise in energy expenditure by an increase in flow rate is especially high for turbulent flow conditions at stenotic valves. In addition, there is an increased risk of arterial rupture by arterial remodelling and an increased risk of thrombosis by deceleration of venous blood flow velocity. Thus, the cardiovascular adaptation to pregnancy means an increased cardiovascular risk which may, on the basis of a basic cardiac disease, lead to cardiac failure.     

Overview of stem cells and imaging modalities for cardiovascular diseases.

Stem cell therapy is emerging as a promising approach to treat heart diseases. Considerable evidence from experimental studies and initial clinical trials suggests that stem cell transplantation promotes systolic function and prevent ventricular remodeling. However, the specific mechanisms by which stem cells improve heart function remain largely unknown. In addition, interpreting the long-term effects of stem cell therapy is difficult because of the limitations of conventional techniques. The recent development of molecular imaging techniques offers great potential to address these critical issues by noninvasively tracking the fate of the transplanted cells. This review offers a focused discussion on the use of stem cell therapy and imaging in the context of cardiology.     

Tissue Doppler imaging a new prognosticator for cardiovascular diseases

Tissue Doppler imaging (TDI) is evolving as a useful echocardiographic tool for quantitative assessment of left ventricular (LV) systolic and diastolic function. Recent studies have explored the prognostic role of TDI-derived parameters in major cardiac diseases, such as heart failure, acute myocardial infarction, and hypertension. In these conditions, myocardial mitral annular or basal segmental (Sm) systolic and early diastolic (Ea or Em) velocities have been shown to predict mortality or cardiovascular events. In particular, those with reduced Sm or Em values of <3 cm/s have a very poor prognosis. In heart failure and after myocardial infarction, noninvasive assessment of LV diastolic pressure by transmitral to mitral annular early diastolic velocity ratio (E/Ea or E/Em) is a strong prognosticator, especially when E/Ea is > or =15. In addition, systolic intraventricular dyssynchrony measured by segmental analysis of myocardial velocities is another independent predictor of adverse clinical outcome in heart failure subjects, even when the QRS duration is normal. In heart failure patients who received cardiac resynchronization therapy, the presence of systolic dyssynchrony at baseline is associated with favorable LV remodeling, which in turn predicts a favorable long-term clinical outcome. Finally, TDI and derived deformation parameters improve prognostic assessment during dobutamine stress echocardiography. A high mean Sm value in the basal segments of patients with suspected coronary artery disease is associated with lower mortality rate or myocardial infarction and is superior to the wall motion score.     

鸟-胞内分枝杆菌复合群肺病和脓肿分枝杆菌肺病的CT影像学比较

目的 探讨鸟胞内分枝杆菌复合群（MAC）肺病和脓肿分枝杆菌肺病的CT影像学特点.方法 回顾性分析2011年1月至2013年10月间在首都医科大学附属北京胸科医院住院并经临床及实验室证实的16例MAC肺病患者和15例脓肿分枝杆菌肺病患者的CT及高分辨率CT（HRCT）的表现.统计学分析采用SPSS13.0软件进行,计数资料采用x2检验,计量资料采用t检验.两组的CT表现、常见病变的分布特点进行四格表x2检验.由于患者总例数＜40例,用确切概率法计算统计结果,以P＜0.05为差异有统计学意义.结果 两组患者中肺实变影（28/31）、支气管扩张（28/31）、小叶中心性结节影及树芽征（28/31）的影像学表现多见.MAC肺病患者上叶发病优势（10/16）高于脓肿分枝杆菌肺病患者（2/15）（确切概率法,P＜0.01）.MAC肺病患者以空洞型为主（11/16）,而脓肿分枝杆菌肺病患者以结节支气管扩张型为主（11/15）（确切概率法,P＜0.05）.MAC肺病患者肺实变、空洞病变、小叶中心性结节影及树芽征累及肺区范围（分别为55/96,33/96,68/96）多于脓肿分枝杆菌肺病患者（分别为26/90,18/90,48/90）（确切概率法,P值分别为＜0.01、＜0.01、＜0.05）.MAC肺病患者于右肺中叶（11/16）及左肺舌叶（12/16）出现肺实变者多于脓肿分枝杆菌肺病（右肺中叶及左肺舌叶均为4/15）（确切概率法,P＜0.05）.结论 鸟-胞内分枝杆菌复合群（MAC）和脓肿分枝杆菌肺病的CT表现有一定相似性,但亦有各自一定的特点,为临床早期诊断及早期治疗可提供一定的帮助.     

Blood pressure, serum cholesterol and smoking habits predicting different manifestations of arteriosclerotic cardiovascular diseases

1661 men aged 40-59 belonging to two rural communities in Italy were enrolled into a longitudinal study in 1960 and some risk factors were measured. In the next 25 years 779 died from all causes, 309 died from arteriosclerotic cardiovascular diseases, and, in particular, 201 from coronary heart disease (CHD), 96 from stroke (STR) and 12 from peripheral arteriosclerotic disease (PAD). The predictive power of mean blood pressure (MBP), of serum cholesterol (CHOL), of cigarette consumption (CIG) and of age (AGE) was evaluated by the multiple logistic model. Beyond AGE, which was always relevant to prediction, the three major risk factors were significant predictors of all cardiovascular diseases (ACVD) and of CHD; only MBP was a significant predictor of STR whereas the limited number of cases prevented from the possibility to obtain stable and significant coefficients for PAD. Within three different sub-categories of CHD only cases ending-up as sudden deaths were significantly related to the three factors including smoking habits, whereas those manifested as myocardial infarction (without sudden death) and those manifested as chronic coronary heart disease (atypical CHD) were related only to MBP and to CHOL.