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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The neonatal lupus syndrome consists of transient cutaneous lupus lesions or permanent congenital complete heart block (or hepatic fibrosis), or both, in infants born to mothers with systemic lupus erythematosus (SLE). The frequency of conduction abnormalities was examined in 86 offspring of 53 women affected by SLE. Electrocardiograms from the offspring demonstrated normal sinus rhythm in 84 of 86 offspring. The PR interval was normal for age (< 95th percentile) in 82 offspring and normal for heart rate in 81. Three children had a PR interval > 95th percentile (i.e., first-degree heart block) for both age and heart rate. The PR interval of the other 6 subjects with first-degree heart block for age or heart rate (> or = 95th percentile) was < or = 0.18 second. In contrast, using a rank assignment of PR intervals in relation to heart rate and age derived from published standards, grouped data indicated that heart rate adjusted for age was greater and PR interval adjusted for heart rate longer in offspring of mothers who had the onset of SLE before or during pregnancy than in the normal population; this observation did not hold for offspring whose mothers developed SLE after the pregnancy. These findings indicate that offspring of mothers with SLE, even in the absence of an abnormal electrocardiogram, may have experienced a maternal internal environment that produces subclinical changes in atrioventricular conduction. However, newborns with a normal pulse rate are unlikely to have significant abnormalities in atrioventricular conduction and do not need screening electrocardiograms at birth.
Am J Cardiol 1993 Jan 01
PMID:Atrioventricular conduction in children of women with systemic lupus erythematosus. 842 Feb 43

The case of a 25-year-old pregnant woman with systemic lupus erythematosus and severe pulmonary hypertension is presented. The pregnancy was complicated by worsening right heart failure and pre-eclampsia, requiring a caesarian section at 29 weeks' gestation. On the fourth day postpartum, the patient's respiratory status worsened and she was transferred to the coronary care unit where she soon died from combined right heart failure and respiratory arrest. The presumed pathogenesis and etiology of lupus-related pulmonary hypertension are discussed, in addition to noninvasive tests and proposed management. Given that the mortality rate is very high during pregnancy and therapy is of limited value, women with lupus-associated pulmonary hypertension should avoid conceiving. Those who choose to become pregnant must be carefully managed by a multidisciplinary team.
Can J Cardiol 1996 Aug
PMID:Systemic lupus erythematosus and pulmonary hypertension during pregnancy: report of a case fatality. 879 79

We describe a case of a verrucous non-bacterial endocarditis on the native mitral valve in a patient with polymyositis. This case, to our knowledge, represents the first report in the literature we could get. The case reached our attention after an episode of acute limb ischemia which lead to an echocardiographic examination that showed vegetations on the valve. No cardiac signs or symptoms were evident. We discuss the possible relation between the two disorders. The possibility of autoimmune diseases, other than lupus or lupus-related disorders, to produce this kind of lesions should be confirmed by a systematic echocardiographic study of these patients even if they have no evidence of cardiac involvement.
G Ital Cardiol 1996 Nov
PMID:[Verrucous abacterial endocarditis and polymyositis. A possible association?]. 903 27

Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels particularly the aorta and its major branches. Recently, the disease has been shown to involve the parenchyma of various organs. Specific glomerular lesions reported in patients with TA are mesangial proliferative, membrano-proliferative, crescentic glomerulonephritis and amyloidosis. Dermatological manifestations of this disease are erythema nodosum, facial lupus rash and erythema induratum. Dilated cardiomyopathy, myocarditis and pericarditis have been reported in TA. Rarely, interstitial lung disease, pneumonic consolidation, idiopathic ulcerative colitis, rheumatoid arthritis and polymyositis have been described in association with TA. In this report, five patients of TA with primary parenchymatous involvement of kidneys, skin, heart and gastrointestinal tract have been described. An association of primary parenchymatous organ involvement and TA suggests an autosensitization to an unidentified antigen in the pathogenesis of TA.
Int J Cardiol 1996 Aug
PMID:Systemic manifestations of Takayasu arteritis: the expanding spectrum. 911 17

This report traces the development of our knowledge about immune-complex arteritis from the early 20th Century to the present time. The emphasis is on the work which began with the seminal observations of serum sickness by Longcope, MacKenzie, and Rich, to the pathogenetic studies of serum sickness arteritis in rabbits by several groups including the outstanding contributions by Dixon and coworkers concerning the role of circulating immune complexes. This work was followed by investigations of the relationship to atherosclerosis revealed by the sustained studies by Minick et al. on serum sickness arteritis in hypercholesterolemic rabbits. This pioneering research work has more recently been of pivotal value in understanding the arteritis observed in certain primate species such as the cynomolgus and the nemestrina, in human lupus erythematosus, and in organ transplantation arteritis. More recently it has become apparent that one of the microscopic hallmarks of this type of immune complex injury is the concentic micro-architecture of the inflammatory arterial lesions, for which, when they are also lipid containing, we have coined the term artheroarteritis. The contributions of the neoantigens from glycosylated LDL and oxidized LDL to the development of this type of atheroarteritis are considered. New frontiers in this area of research are being opened by the PDAY study which offers new opportunities to link circulating immune complexes and new antigens to arheroarteritis with its accelerated stenotic arterial lesion development.
Int J Cardiol 1996 Aug
PMID:Atheroarteritis: a combined immunological and lipid imbalance. 911 26

Early diagnosis and greatly improved treatment have markedly altered the clinical evolution of systemic lupus erythematosus; the pattern of cardiac involvement in lupus has also changed. To illustrate this, a young woman who died from severe mitral valve disease, including a coronary embolus from verrucous endocarditis, is presented. Mitral valve involvement in lupus is no longer limited to the small benign lesions described by Libman and Sacks.
Can J Cardiol 1997 Sep
PMID:Lupus-related mitral valve disease: embolic coronary occlusion as a unique cause of myocardial infarction. 934 38

To examine whether primary hypercoagulable states, such as protein C and protein S deficiencies and antiphospholipid antibody, are associated with an increased risk, severity and recurrence of acute pulmonary thromboembolism, the blood levels of antithrombin III, protein C, protein S, lupus anticoagulant, anticardiolipin antibodies were measured in 23 patients with acute pulmonary thromboembolism, 7 men and 16 women (mean age +/- SD, 52 +/- 5 years), admitted to Nippon Medical School Hospital from January, 1990 through December, 1997. Four patients (17.4%) had protein C deficiency, one had protein S deficiency (4.3%), 10 had lupus anticoagulant (43.5%), and 2 had anticardiolipin antibodies (8.7%). Hemodynamic evaluation by Swan-Ganz catheter and venography of the lower extremity to detect deep venous thrombi were carried out in 20 and 21 patients, respectively. There were no differences between the patients with and without primary hypercoagulable states in age (52 +/- 14 vs 52 +/- 19 years), gender, or percentage of patients with deep venous thrombi in the lower extremity (91.7% vs 88.9%). Mean pulmonary arterial pressure (38 +/- 9 vs 26 +/- 4 mmHg, p < 0.05) and total pulmonary vascular resistance (10 +/- 5 vs 6 +/- 2 Wood unit, p < 0.1) were higher in the 13 patients with primary hypercoagulable states compared with the 10 patients without primary hypercoagulable states. Recurrence of pulmonary thromboembolism tended to be higher in the 13 patients with primary hypercoagulable states compared with the 10 patients without primary hypercoagulable states (46.2% vs 10.0%, p < 0.1). These findings suggest that primary hypercoagulable states, such as protein C and S deficiencies and antiphospholipid antibody, are associated with the severity and increased risk and recurrence of acute pulmonary thromboembolism.
J Cardiol 1998 Oct
PMID:[Potential role of primary hypercoagulability and antiphospholipid antibody as a risk factor of acute pulmonary thromboembolism]. 983 33

The history of antiarrhythmic therapy reveals these agents to be associated with a high incidence of toxicity. Although several agents have ocular effects, amiodarone is the most widely recognized for producing adverse effects in the eyes. Corneal microdeposits are almost ubiquitous in patients being treated with amiodarone. However, they are, for the most part, benign and produce no changes in visual acuity. Lack of microdeposits should prompt the physician to investigate whether there is a problem with drug absorption or adherence to therapy. Other effects on the eye have been reported including optic neuropathy, but no causal link has been proved with amiodarone. The population of patients treated with amiodarone often have ischemic disease and/or diabetes, which affect retinal and optic nerve health. Many antiarrhythmic agents also affect lung function. The frequent association of procainamide with a lupus-like syndrome, where half the cases develop pleural-pericardial involvement, may require discontinuation of that drug. Although beta blockers and to a lesser degree, calcium antagonists, may cause bronchospasm in some patients, this is not usually a major clinical problem. Again, it is amiodarone that has the most widespread reputation for causing pulmonary toxicity. Although infrequent (< 1% incidence), it generates the most fear as it is sometimes fatal. Because of the lack of a diagnostic "gold standard," it is often overdiagnosed, placing patients at risk from overlooked congestive heart failure and infections and from recurrent arrhythmias after drug withdrawal. Patients with pre-existing pulmonary disease appear to be more at risk. Common features include indolent onset of cough, malaise and fever associated with patchy peripheral infiltrates, and severely decreased diffusion capacity. Several cases of pulmonary toxicity have had inordinately high serum desethylamiodarone to amiodarone ratios. Most cases recover with cessation of amiodarone therapy. Steroids are commonly used, but are of unproved efficacy. In terms of its toxicity, amiodarone remains the most feared of the antiarrhythmic agents. In the future, a better understanding of its pharmacokinetics, mechanisms of toxicity, and optimal dosing regimens should provide a possibility of better strategies for avoidance, early diagnosis, and more directed therapy of toxicities associated with amiodarone.
Am J Cardiol 1999 Nov 04
PMID:Clinical organ toxicity of antiarrhythmic compounds: ocular and pulmonary manifestations. 1056 58

MRL/MpJ-lpr/lpr (MRL/lpr) mice spontaneously develop immune complex-mediated glomerulonephritis, granulomatous arteritis, and thrombocytopenia. Recent genetic analyses in a few different strains of lupus-prone mice have pointed out a close correlation between autoantibodies reactive with the endogenous retroviral env gene product, gp70, and the development and severity of glomerulonephritis. We have also shown that autoantibodies reactive with endogenous retroviral gp70 are closely correlated with the development of necrotizing polyarteritis in another lupus-prone strain of mice, SL/Ni. However, suggested pathogenicity of anti-gp70 autoantibodies has not yet been directly tested. To examine if anti-gp70 autoantibodies induce glomerular and vascular pathology, we established from unmanipulated MRL/lpr mice hybridoma clones that secrete monoclonal antibodies reactive with endogenous xenotropic viral env gene products. As reported separately, a high proportion of these anti-gp70 antibody-producing hybridoma clones induced in syngeneic non-autoimmune and severe combined immunodeficiency mice proliferative or wire loop-like glomerular lesions with granular deposits of gp70, IgG, and C3 in affected glomeruli. Some mice transplanted with these anti-gp70 autoantibody-producing hybridoma cells also showed massive subendothelial deposition of electron-dense materials in small arterioles in the kidneys. Furthermore, we identified an IgG2a-producing anti-gp70 hybridoma clone that induced microvascular intraluminal platelet aggregation, thrombocytopenia, and amenia upon transplantation into syngeneic non-autoimmune mice. This anti-gp70 autoantibody bound onto the surfaces of mouse platelets, and specifically precipitated a platelet protein with an approximate relative molecular mass of 40000. Attachment of activated platelets to the intimal surfaces of small arteries was also observed by electron microscopy in mice transplanted with the pathogenic anti-gp70 IgG2a-producing hybridoma cells, suggesting an interaction between antibody-bound platelets and endothelial cells.
Int J Cardiol 2000 Aug 31
PMID:Roles of endogenous retroviruses and platelets in the development of vascular injury in spontaneous mouse models of autoimmune diseases. 1098 Mar 39

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 +/- 7 vs 51 +/- 4 beats/min, p = 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.
Pediatr Cardiol
PMID:Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases. 1152 12


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