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

Myocardial involvement in lupus erythematosis takes the form of an interstitial myocarditis with cellular infiltration and fibrinoid necrosis. The most lesions are perivascular, and involve the arterioles. The myocardial fibres are involved secondarily to the vascular lesions, or by grossly, damaging sclerosis. The clinical features are variable:--no clinical features, but haemodynamic evidence of abnormal ventricular function, and perhaps sudden death;--arrhythmias and disorders of atrio-ventricular conduction;--cardiac failure, which may be due to a genuine cardiomyopathy (a part may be played by hypertension, pulmonary hypertension, renal failure, constrictive pericarditis or haemodynamically major valve disorders);--abnormalities of the coronary trunk in a certain number of cases. If anti-nuclear antibodies are present in a cardiomyopathy, the presence of DLE or of a drug-induced lupus syndrome must be suspected. There remain some awkward cases which defy classification, and which systematic use of echocardiography and pericardial and myocardial biopsy may be able to define more accurately.
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PMID:[The myocardiopathies of systemic lupus erythematosus]. 9 56

The natural history of the cardiovascular manifestations of systemic lupus erythematosus (SLE) have been altered by corticosteroids which exert their own cardiovascular effects. This study describes clinical and necropsy observations in 36 corticosteroid-treated patients with SLE and compares them to necropsy observations in patients with SLE reported before the use of corticosteroid therapy. The 36 patients averaged 32 years of age, and 33 were women. Systemic hypertension was present in 25 (69 per cent) and left ventricular hypertrophy in 23 (64 per cent) patients. Hypertension was twice as common in the 19 patients who received this drug for more than 12 months (average 38 months) than in the 17 patients who received this drug for less than 12 months (average 6 months), and was almost five times more common among our patients than in patients with SLE in the presteroid era. Congestive cardiac failure occurred in 15 patients (43 per cent), eight times more frequent than that reported in noncorticosteroid-treated patients with SLE. Subepicardial and myocardial fat was increased in all 36 patients. Lupus carditis was similar in frequency but differed morphologically in our patients compared to those not treated with corticosteroids. Libman-Sacks-type endocardial lesions, present in 18 (50 per cent) of our patients, were smaller, fewer in number, univalvular rather than multivalvular, and mainly left-sided. Most verrucae were either partly or completely healed, and some were calcified. Pericarditis, present in 19 (53 per cent) patients, was predominantly of the fibrous type. Myocarditis was present in three patients, each of whom also had endocarditis and pericarditis. The lumen of at least one of the three major coronary arteries was narrowed more than 50 per cent by atherosclerotic plaques in 42 per cent of the 18 patients who received corticosteroids for more than 1 year, but in none of the 17 patients who received corticosteroids for less than 1 year. Four of the eight patients with narrowed coronary arteries had myocardial infarcts. Although vital to the management of SLE, corticosteroids have an over-all deleterious effect on the heart. Systemic hypertension and left ventricular hypertrophy appear or, when present, worsen; congestive cardiac failure increases; epicardial apartment of Me
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PMID:The heart in systemic lupus erythematosus and the changes induced in it by corticosteroid therapy. A study of 36 necropsy patients. 111 70

Neonatal lupus is a model of passively acquired autoimmunity in that immune abnormalities in the mother lead to the production of antibodies that cross the placenta and injure the developing fetus. Congenital complete heart block (CCHB), a permanent manifestation of neonatal lupus, is detectable after 18 wk gestation. Transient manifestations include cutaneous, hepatic, and hematologic abnormalities that occur at variable frequency. To date, there is a universal association of CCHB with maternal antibodies to SSA/Ro-SSB/La ribonucleoproteins, detectable by high ratio monomer:crosslinker SDS-immunoblot. Intriguingly, cardiac disease and often other manifestations are not present in the mother, raising the hypothesis that there is differential expression and/or accessibility of SSA/Ro-SSB/La antigens in fetal vs. adult tissues. CCHB may be a final consequence of a more widespread inflammatory response in the heart, including the existence of an associated myocarditis. In contrast to the in utero onset of CCHB, skin lesions generally become apparent after birth. Ultraviolet exposure may be an initiating factor and exacerbate an existing rash. Several studies have documented the predominance of DR3 alleles in mothers of affected offspring, frequently associated with the extended haplotype A1,B8. Available evidence suggests that fetal genetic differences in the major histocompatibility complex (MHC) do not influence susceptibility. The recommended clinical approach includes obstetric and rheumatologic management of both the fetus identified with CCHB and the fetus with a normal heart beat but at high risk of developing CCHB. Fetal echocardiogram is essential in diagnosing and following disease and may suggest the presence of an associated myocarditis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Neonatal lupus syndromes. 128 97

Coronary artery disease has emerged as an important cause of death in young patients with SLE. We report three cases of acute myocardial infarction in young lupus patients who underwent emergent coronary angiography. One patient had a large coronary aneurysm and died five months later from myocarditis. The other two patients underwent coronary angioplasty. The difficulty in distinguishing coronary arteritis from premature atherosclerosis and its relevance to methods of treatment is discussed.
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PMID:Evaluation and treatment of acute myocardial infarction complicating systemic lupus erythematosus. 173 66

Over the last 10 years, our knowledge of immunologically mediated processes involving the myocardium appears to have made quantum leaps. New and important disease entities such as AIDS have appeared and the cardiologist now becomes an important member of the "AIDS team." Our understanding of "older diseases" such as sarcoidosis, Lyme disease, systemic lupus and other connective tissue syndromes has significantly increased. The concept of high-dose steroid therapy for these processes may, in fact, turn out to be futile and more selective, as less dangerous immunosuppression is being introduced. This concept has significantly advanced in the field of cardiac transplantation where immunosuppression has now been usurped by specific immunotherapy aimed at selective aspects of the immune sequence. New and exciting concepts will emerge from the molecular biology laboratory that will have direct bearing on the management of patients with cardiovascular disorders. This information explosion will force the cardiovascular physician to become more in tune with the world of immunology and molecular biology. Many obvious, significant problems remain, such as accelerated atherosclerosis in the transplant patient and the role of myocarditis in the patient with heart failure. However, it will truly be an exciting decade in which to work and watch the unraveling of these mysteries and hopefully, the study of today's problems will give way to solutions and a clearer understanding of the heart as a target of immune injury.
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PMID:The heart as a target organ of immune injury. 191 12

We report on a newborn with fetal acquired heart block (CHB). CHB is a rare, irreversible defect, commonly occurring in conjunction with myocarditis in the neonatal lupus syndrome. Development of CHB is strongly associated with maternal anti-SS-A(Ro)/SS-B(La) antibodies. Intrauterine therapy of CHB is not possible. Concomitant myocarditis, however, can be treated effectively with dexamethasone. Mothers with an elevated risk of fetal CHB can be identified by their history (underlying systemic connective tissue disease, previous pregnancies with CHB), an immunogenetic predisposition (HLA-DR3) and analysis of the SS-A/SS-B antibody pattern. In these pregnancies prevention of CHB with plasmapheresis plus dexamethasone during pregnancy may be possible.
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PMID:[Fetal complete heart block with myocarditis and maternal SS-A-/AA-B/antibodies]. 225 94

Since systemic lupus erythematosus most frequently affects women of childbearing years, the management of patients during pregnancy is an important and common problem facing the clinician. This review concerns the effects of pregnancy on the course of maternal disease and fetal well-being. On the maternal side are the problems of renal disease which may exacerbate and be difficult to differentiate from pre-eclampsia especially when occurring in the third trimester. An active urinary sediment, falling C3 and CH50 and elevated complement split products of the alternative pathway and terminal attack complex may serve as useful parameters of lupus activity. In general, maternal disease is not an imposing threat and prospective studies suggest that the exacerbation rate is not significantly greater in the pregnant lupus patient than in the non-pregnant patient. On the fetal side are the problems of placental insufficiency and in utero attack on developing organs. Maternal antibodies such as those reactive with negatively charged phospholipids are associated with second trimester miscarriages and suggested, but not firmly established, thrombosis of placental vessels. The placental transfer of maternal antibodies against components of the rapidly expanding group of SSA/Ro-SSB/La ribonucleoproteins is strongly implicated in the transient and permanent manifestations of neonatal lupus. Using various techniques for defining the specificity of the antibody response most associated with heart block, the data suggest that mothers whose sera contain antibodies which recognize antigens of SSA/Ro-SSB/La on SDS-immunoblot are at greatest risk. In the absence of antibodies to SSB/La, mothers whose sera contain antibodies reactive only to bovine SSA/Ro by ELISA do not appear to be at high risk. A rational approach to in utero treatment of autoantibody mediated fetal myocarditis includes plasmapheresis and the use of dexamethasone. Finally, the safety of the commonly used medications for the treatment of lupus such as the nonsteroidal anti-inflammatory agents, glucocorticoids and anti-malarials during gestation and breast feeding, is addressed.
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PMID:Systemic lupus erythematosus and the maternal-fetal dyad. 228 64

This is a case of a child with neonatal lupus and congenital atrioventricular (AV) block, born to a mother with asymptomatic, systemic lupus erythematosus (SLE). The child, despite pacemaker insertion, died of septicemia and myocarditis at the age of three months. Although the association of neonatal lupus with congenital AV block is well-recognized, there are only few pathologic studies of the conduction system reported in the literature. This is such a study in which we emphasize that, due to an altered immune system in the child, septicemia may be the cause of death in some cases.
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PMID:Neonatal lupus with congenital atrioventricular block and myocarditis. 244 30

Antibodies to Extractable Nuclear Antigens (ENAs) namely Sm, nRNP, SS-A and SS-B were studied in 397 patients with various connective tissue diseases (CTD), 146 patients with inflammatory polyarthropathies, 16 cases of systemic vasculitides, and 39 normal subjects using counterimmunoelectrophoresis and double immunodiffusion methods. Anti-ENA antibodies were positive in 40.8 percent cases of Systematic lupus erythematosus (SLE) (n = 191), 36.4 percent of overlap CTD (OCTD, n = 44), 27.8 percent of Sjogren's syndrome (n = 18), 10.6 percent of progressive systemic sclerosis (PSS, n = 66) and 2.7 percent of rheumatoid arthritis (n = 111) patients. The correlation of these antibodies with disease features was done. The significant finding was negative association of anti-nRNP antibodies (when present alone) with renal involvement. Anti-Sm antibodies did not correlate with any disease feature. The other associations included correlation of anti-nRNP with pulmonary parenchymal lesions, anti-SS-A with serositis and pulmonary hypertension, and anti-SS-B with myocarditis and recurrent diarrhoea. We conclude that Anti-ENAs may correlate with certain subsets of these diseases but the subject is controversial.
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PMID:Antibodies to extractable nuclear antigens in connective tissue disorders in India: prevalence and clinical correlations. 262 64

The neonatal lupus syndromes, which comprise transient hematologic and cutaneous disorders as well as the permanent manifestation of heart block, are considered to result from injury by passively acquired maternal autoantibodies. The active placental transport of maternal IgG antibodies becomes operative late in the second trimester coincident with the time at which bradycardia and myocarditis become evident. Surprisingly there are no clinically detectable abnormalities in the maternal hearts. The recognition that antibodies to the SSA/Ro-SSB/La ribonucleoprotein complex were found in 85% of sera from mothers of offspring with neonatal lupus was an important advance and directed attention to these antigens as potential candidates despite their intracellular location. In the present review we describe an experimental approach to the treatment of a fetus diagnosed by in utero echocardiogram to have congenital complete heart block and to the prevention of this condition in an at-risk pregnancy. In an attempt to more specifically define the relevant antigen-antibody systems involved in the pathogenesis of neonatal lupus we have utilized the technique of immunoblot to evaluate sera from mothers of offspring with permanent manifestations of neonatal lupus including heart block and hepatic fibrosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Neonatal lupus and congenital complete heart block: manifestations of passively acquired autoimmunity. 269 Nov 58


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