Cardiac involvement in rheumatic diseases

In the enclosed paper cardial changes of different rheumatic diseases are concerned. It refers to the change concerning the cardial symptoms of the single diseases and differential diagnostic considerations are made. Essential cardial symptoms and important symptoms and therapeutic possibilities are named.     

几种风湿性疾病的心脏表现

1　类风湿关节炎 (RA)类风湿关节炎是一种原因不明、以慢性进行性关节病变为主的自身免疫性疾病 ,除关节病变外 ,伴随全身多系统损害 ,心血管系统受损也是类风湿关节炎重要的关节外表现之一。急性期类风湿关节炎可引起心脏损害 ,为全心炎 ,包括心包、心肌、心内膜、传导阻滞 ,严重的冠状动脉炎可导致心肌梗死。心包炎常见 ,可发生于疾病的任何时期 ,早期及病情活动时多见 ,常伴有类风湿结节、血管炎、类风湿因子 (RF)阳性。心包炎常与胸膜炎同时发生。尸检发现 30 % - 6 0 %有心包受累 ,超声心动图检查 30 %的患者可有心包积液 ,患者可无…     

Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases

Rhythm and conduction disturbances and sudden cardiac death (SCD) are important manifestations of cardiac involvement in autoimmune rheumatic diseases (ARDs). In patients with rheumatoid arthritis (RA), a major cause of SCD is atherosclerotic coronary artery disease, leading to acute coronary syndrome and ventricular arrhythmias. In systemic lupus erythematosus (SLE), sinus tachycardia, atrial fibrillation and atrial ectopic beats are the major cardiac arrhythmias. In some cases, sinus tachycardia may be the only manifestation of cardiac involvement. The most frequent cardiac rhythm disturbances in systemic sclerosis (SSc) are premature ventricular contractions (PVCs), often appearing as monomorphic, single PVCs, or rarely as bigeminy, trigeminy or pairs. Transient atrial fibrillation, flutter or paroxysmal supraventricular tachycardia are also described in 20-30% of SSc patients. Non-sustained ventricular tachycardia was described in 7-13%, while SCD is reported in 5-21% of unselected patients with SSc. The conduction disorders are more frequent in ARD than the cardiac arrhythmias. In RA, infiltration of the atrioventricular (AV) node can cause right bundle branch block in 35% of patients. AV block is rare in RA, and is usually complete. In SLE small vessel vasculitis, the infiltration of the sinus or AV nodes, or active myocarditis can lead to first-degree AV block in 34-70% of patients. In contrast to RA, conduction abnormalities may regress when the underlying disease is controlled. In neonatal lupus, 3% of infants whose mothers are antibody positive develop complete heart block. Conduction disturbances in SSc are due to fibrosis of sinoatrial node, presenting as abnormal ECG, bundle and fascicular blocks and occur in 25-75% of patients.     

Cardiac imaging techniques in systemic autoimmune diseases

Systemic autoimmune disorders are frequently associated to cardiac involvement and to a high prevalence of ischemic coronary events, often occurring at a younger age than in the normal population. Large increase in mortality is related to premature atherosclerosis with coronary artery disease and stroke in patients with connective tissue diseases. Coronary heart disease is responsible for 40-50% of the deaths of patients with rheumatoid arthritis. Transesophageal or transthoracic echocardiography are the most useful and noninvasive techniques able to detect not only valvular abnormalities, embolic sources or pulmonary hypertension, but also left ventricular systolic or diastolic dysfunction. Furthermore, the introduction of new indexes, contrast agents and software increased the accuracy of this technique. It is possible now to evaluate coronary flow reserve by transthoracic echocardiography in patients with systemic autoimmune disease in order to detect microvasculature disorder. However, an ischemic response in a symptomatic patient requires, in most cases, further evaluation with cardiac catheterization. Coronary artery imaging allows confirmation of the presence, extent and position of atheromatous lesions. More recently, other imaging modalities including magnetic resonance and computerized tomography angiography have been developed to allow imaging of the coronary arteries.     

Magnetic resonance imaging of peripheral joints in rheumatic diseases

The need for better methods than the conventional clinical, biochemical and radiographical examinations in the management of inflammatory joint diseases is evident, since these methods are not sensitive or specific to early pathologies and subtle changes. Magnetic resonance imaging (MRI) offers improved sensitivity to early inflammatory and destructive changes in peripheral joints in rheumatoid arthritis (RA) and, even though less well documented, in other inflammatory joint diseases. Good evidence is available that MRI bone erosions represent true bone abnormalities and are predictors of radiographical outcome in RA. Similarly, there is solid evidence for MRI synovitis representing true synovial inflammation and being of considerable practical, clinical and radiological significance in RA. Describing the encouraging current knowledge regarding MRI for diagnosis, monitoring and prognosis, this chapter discusses the potential for the use of MRI in the clinical management of patients with suspected and diagnosed inflammatory joint diseases, as well as research priorities and clinical situations where the use of MRI could be suggested.     

Use of imaging in the differential diagnosis of rheumatic diseases in children

Imaging studies are important adjuncts in the evaluation of children with suspected rheumatic diseases. They are best used as follow-up investigations to exclude specific differential diagnoses, based upon a careful history and physical examination. Often, repeated examination over a period of time, sometimes months, is necessary before a diagnosis can be made. The addition of selected imaging studies can be important in the common circumstance where no diagnosis is made, but the physician must assure the child and family that all appropriate efforts have been made to exclude important illnesses.     

New developments in imaging for diagnosis and therapy monitoring in rheumatic diseases

The availability of therapeutic modalities that are able to stop inflammatory joint damage has also markedly influenced recent developments in muskuloskeletal imaging. One focus of interest is the detection of joint pathology as early as possible in order to prevent erosive bony changes. Ultrasonography and magnetic resonance imaging are the most valuable technologies in this respect. Another focus is on the exact assessment and documentation of joint damage using scoring systems not only in therapeutic trials, but also in clinical practice. In addition to these recent advances in peripheral and axial joint imaging, this chapter also discusses advances in vascular imaging and scintigraphy in rheumatoid diseases as well as interventional procedures guided by imaging technologies.     

Scintigraphy in rheumatic diseases

The aim of this review is to summarise the clinical role of nuclear medicine in rheumatology taking into consideration the most specific diagnostic applications and other worthwhile therapeutic contributions. Traditional bone scintigraphy and recent inflammation-targeting radiopharmaceuticals, such as radiolabelled leucocytes and immunoscintigraphy, now allow us to obtain highly sensitive total-body and tomographical imaging information that can be used for the diagnosis of osteoarticular disease. The most common extra-articular manifestations of rheumatic diseases due to digestive, central nervous, respiratory and cardiovascular system involvement can be diagnosed by specific scintigraphic methods. Radiosynovectomy plays an important role in providing effective treatment for some joint diseases that are resistant to pharmacological therapy. Diagnostic and therapeutic applications of nuclear medicine show the highest efficacy in the early phase of rheumatic diseases. In more advanced stages, scintigraphical techniques play a complementary role to radiographical investigations in the assessment of prognosis and therapy efficacy.     

Epigentics in rheumatic diseases

The human genome comprises approximately 30000 genes needed for the formation and function of approximately 1 Million proteins in the human body. Differentiation leads to the deactivation of genes that are not needed in the specific tissues or cells. To regulate the cell specific gene expression in normal cells epigenetic modifications work in concert with genetic mechanisms. In contrast to genetic mutations, epigenetics encompasses the wide range of heritable changes in gene expression that do not result from alteration in the DNA sequence itself. A dysregulation of epigenetic modifications results in diseases such as cancer or autoimmune diseases. Since these epigenetic modifications of the DNA and the histones are reversible they are good targets for novel therapeutic intervention.     

Calcinosis in rheumatic diseases

BACKGROUND: Calcinosis, or dystrophic soft-tissue calcification, occurs in damaged or devitalized tissues in the presence of normal calcium/phosphorus metabolism. It is often noted in the subcutaneous tissues of connective tissues diseases--primarily systemic lupus erythematosus, scleroderma, or dermatomyositis--and may involve a relatively localized area or be widespread. The calcinotic accumulations may lead secondarily to muscle atrophy, joint contractures, and skin ulceration complicated by recurrent episodes of local inflammation and infection. OBJECTIVES: To review the classification, pathogenesis, clinical features, and treatment of calcinosis in rheumatic diseases. METHOD: A MEDLINE search of articles from 1972 to 2004 was conducted utilizing the index word "calcinosis" with the coindexing terms "scleroderma," "lupus," "dermatomyositis," and "dystrophic calcification." RESULTS: Calcinosis may be the source of both pain and disability in connective tissue disease patients. Illustrative cases of patients with severe calcinosis are described. The literature available was critically reviewed. While warfarin, colchicine, probenecid, bisphosphonates, diltiazem, minocycline, aluminum hydroxide, salicylate, surgical extirpation, and carbon dioxide laser therapies have been used, no treatment has convincingly prevented or reduced calcinosis. CONCLUSIONS: Calcinosis is common in the conditions reviewed and a number of agents have been used for treatment. However, the approach to calcinosis management is disorganized, beginning with the lack of a generally accepted classification and continuing with a lack of systematic study and clinical therapeutic trials.     

Selenium in rheumatic diseases

Selenium is involved in several important biochemical pathways relevant to rheumatic diseases. Experimental and clinical studies suggest that selenium modulates the inflammatory and immune responses. Patients suffering from inflammatory rheumatic diseases often have low selenium levels, but this finding does not correlate with disease severity. Selenium supplementation needs stricter selection criteria and better ascertainment of dose to obtain a stimulatory or inhibitory effect relevant to the disease state. Prevention of marginal selenium deficiency by moderate supplementation might enhance host defense mechanisms.     

Lysozyme in rheumatic diseases

Lysozyme activity was determined in 235 sera, 258 synovial fluids, and 91 urines of patients with various articular diseases. In the serum, increased activity of the enzyme was observed in 34% of patients with rheumatoid arthritis and in a proportion of patients with gout. In general, serum lysozyme was normal in osteoarthritis and other articular diseases.     

Osteoporosis in rheumatic diseases

Rheumatic diseases, characterized by chronic inflammation and damage to various organs and systems, include systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis and other connective tissue diseases. Bone is a target in many inflammatory rheumatic diseases. In recent years, the survival of patients with rheumatic diseases has increased markedly and the relationship between rheumatic diseases and osteoporosis (OP) has become more prominent. OP and related fragility fractures increase the morbidity and mortality of rheumatic disease. The cause of OP in rheumatic diseases is complex. The pathogenesis of OP in rheumatic diseases is multifactorial, including disease and treatment-related factors. Osteoimmunology, a crosstalk between inflammatory and bone cells, provides some insight into the pathogenesis of bone loss in systematic inflammatory diseases. The aim of this article is to review different risk factors in rheumatic diseases. Several factors play a role, such as chronic inflammation, immunological factors, traditional factors, metabolism and drug factors. Chronic inflammation is the most important risk factor and drug treatment is complex in patients with OP and rheumatic disease. Attention should be paid to bone loss in rheumatic disease. Optimal treatment of the underlying rheumatic disease is the first step towards prevention of OP and fractures. Apart from that, a healthy lifestyle is important as well as calcium and vitamin D supplementation. Bisphosphonates or denosumab might be necessary for patients with a low T score.