Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Complete heart block was diagnosed prenatally in 21 fetuses. Associated structural cardiac defects were present in 18 fetuses, in particular complete atrioventricular canal with atrial isomerism (5 cases), and 'corrected' transposition of the great arteries (4 cases). Maternal systemic lupus erythematosus was proved in only one case. In 11 fetuses, intra-uterine congestive heart failure with the signs of non-immune hydrops fetalis occurred. In all 11 fetuses, the hydrops was associated with a cardiac defect, in particular complete atrioventricular canal with atrial isomerism in 5 cases. A review of the literature confirms that only the association of complete heart block and cardiac malformation can cause intra-uterine congestive heart failure, whereas in the case of fetal complete heart block without cardiac malformation or with prenatally hemodynamically insignificant cardiac malformation, congestive heart failure is rare. Only 30% of newborns with complete heart block have associated cardiac malformations. In our series, however, 86% of the fetuses with complete heart block had cardiac malformations. The most important reason for this percentage discrepancy is that almost all fetuses with associated severe cardiac defects, in particular atrioventricular canal defects, develop heart failure which frequently results in prenatal death. Thus, fetal deaths are not included in pediatric statistics. Nevertheless, fetuses with isolated complete heart block generally do not develop heart failure and in almost all of the cases are born alive.
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PMID:Fetal complete heart block: antenatal diagnosis, significance and management. 265 98

The neonatal lupus erythematosus syndrome, first described by McCuistion and Schoch in 1954, is associated with characteristic skin lesions and congenital heart block in the new-born, and the presence of Ro-(SSA), La-(SSB), or RNP antibodies in mothers and infants. A transplacental transference of maternal autoantibodies is discussed as possible pathophysiologic mechanism in neonatal lupus. The symptoms, the onset, and recently published pathogenetic concepts are reviewed.
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PMID:[Neonatal lupus erythematosus]. 266 Apr 70

Experience with more than 150 pregnancies of women with systemic lupus erythematosus demonstrates that: many conventional measures of lupus activity, including complement, platelet count and urinary protein, are invalid during pregnancy; pregnancy does not cause lupus exacerbation; anti-phospholipid antibody is common and is closely associated with fetal loss, but is not the sole determinant factor of fetal loss; specific characteristics of anti-phospholipid antibody do not identify which antibody-positive women will have poor fetal outcome; prednisone therapy does not improve fetal prognosis; and neonatal lupus, diagnosed by rash and thrombocytopenia, is common but congenital heart block is rare.
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PMID:Pregnancy in systemic lupus erythematosus. 269 Nov 57

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

The literature about pregnancies in patients with systemic lupus erythematosus (SLE) was reviewed. Information about 1,164 pregnancies was compiled. Fifty-four per cent of the pregnancies were normal, 15 per cent of the babies were premature, and 29 per cent of the pregnancies resulted in fetal wastage. In a background population of 50,000 women the corresponding figures were 80, 4 and 16 per cent, respectively. The neonatal mortality of babies borne by women with SLE was 2 per cent. About one third of the patients had an exacerbation of their disease, mostly post-partum or during the last trimester of the pregnancy. The risk of a exacerbation for patients with active disease at the time of conception was double that of patients with inactive disease. Therapeutic abortions were followed by improvement as well as deterioration of the disease. Two per cent of the patients died during pregnancy or post-partum. Cardiolipin-antibody may be a marker of fetal wastage and Ro-antibody may be a marker of fetal heart block in babies borne by women with SLE. Pregnancy was previously considered inadvisable in all patients with SLE. During recent years, the attitude to this problem has been less rigid. However the patients should be fully informed about the risk of exacerbation, fetal wastage, and premature delivery and patients with active disease should be advised to postpone pregnancy until a remission has been achieved.
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PMID:[Systemic lupus erythematosus and pregnancy]. 273 64

The molecular basis of autoantibody reactivity with components of the SSA/Ro-SSB/La particle exhibited by sera of mothers of infants with severe and permanent manifestations of neonatal lupus (NLE) was investigated using immunoblotting and immunoprecipitation. The characteristics of NLE that were studied included congenital complete heart block (CCHB), second degree heart block, and hepatic fibrosis. Antibodies specific for one or more components of the SSA/Ro-SSB/La particle were found in sera from all 20 mothers of permanently affected infants. However, no antibody specific for a single peptide of this particle was common to all sera. Using tissue extracts from a human cell substrate, 80% of these sera had antibodies to one or more components of the SSA/Ro particle demonstrable by immunoblotting. The predominant antibody response in the NLE group was to the newly recognized 52-kD SSA/Ro peptide component. In contrast, antibodies to the 60-kD SSA/Ro component although present, were the least represented and not significantly increased in frequency among mothers of these infants, compared with a group of 31 mothers with autoimmune diseases such as systemic lupus erythromatosus (SLE) but who had healthy offspring. Antibodies directed to the 48-kD SSB/La antigen were demonstrated in 90% of the NLE mothers often accompanying antibodies against the 52-kD SSA/Ro component. The combination of antibodies to 48- and 52-kD structures was significantly increased in the NLE group, with an odds ratio of 35. The type of cell or tissue substrate was shown to influence detectability of antibodies. The 52-kD SSA/Ro peptide and the 48-kD SSB/La peptide were abundant in cardiac tissues from fetuses aged 18-24 wk, further supporting the possible relevance of these peptides to heart block.
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PMID:Acquired congenital heart block. Pattern of maternal antibody response to biochemically defined antigens of the SSA/Ro-SSB/La system in neonatal lupus. 276 Feb 4

The neonatal lupus syndrome is the most common cause of isolated congenital heart block. There is, at present, little information about the putative role of anti-SS-A/Ro SS-B/La antibodies in the pathogenesis of congenital heart block in the neonatal lupus syndrome. Using an in vitro experimental model, the present study was designed to test the hypothesis that IgG antibodies in the sera of anti-SS-A/Ro SS-B/La-positive mothers of infants with isolated congenital heart block bind to and affect the transmembrane action potential of rabbit cardiac tissue. The results demonstrate a preferential inhibition of membrane repolarization (ADP-50 and ADP-90) and staining of cardiac cells within the neonatal, in contrast to the adult, rabbit heart by sera and IgG-enriched fractions from anti-SS-A/Ro SS-B/La-positive individuals. The results of the electrophysiologic studies demonstrate a pathophysiologic role for the IgG fraction of anti-SS-A/Ro SS-B/La-positive maternal sera in inhibiting neonatal rabbit cardiac repolarization. It is possible that antibodies to similar determinants expressed on the cell membrane of cardiac-conducting cells also may play a pathophysiologic role in the development of idiopathic congenital heart block in humans.
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PMID:Anti-SS-A/Ro SS-B/La antibodies bind to neonatal rabbit cardiac cells and preferentially inhibit in vitro cardiac repolarization. 278 47

The neonatal lupus syndrome and congenital heart block are strongly associated with the presence of antibodies to the ribonucleoprotein antigen Ro. The study of these conditions has given insight into possible pathogenetic mechanisms operating in connective tissue diseases.
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PMID:The neonatal lupus syndrome. 305 68

Systemic lupus erythematosus (SLE) is a disease occurring mostly in women in the childbearing age. We present two cases of pregnancy complicated one by SLE, the other by a "lupus-like" syndrome. In the first case, the fetus suffered from a complete heart block; in the second one, a lupus anticoagulant was discovered in the mother. These two cases are discussed by the light of the literature.
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PMID:[Lupus erythematosus, circulating anticoagulant and obstetric complications. Apropos of 2 cases]. 310 57

Both maternal isoimmunization and maternal autoimmune disease are associated with fetal death. For isoimmunization the immunologic nature of fetal death (hydrops fetalis) is beyond question, but many of the details are poorly understood. It would be extremely helpful to know what immunologic factors are responsible for the wide variation in the degree of fetal hemolysis. This information would surely lead to improved management of isoimmunized pregnancies and create new and more successful therapies for fetuses at risk for hemolysis. The immunology of autoimmune-associated fetal death is, for the most part, an enigma. For the fetal deaths associated with SLE and the antiphospholipid antibodies, demise appears to be a consequence of uteroplacental vascular damage. But the observable pathology is nonspecific, and the evidence for a direct immunologic mechanism is sparse. The similarity between the uteroplacental vascular lesions found with these autoimmune conditions and those seen in preeclampsia demands more intensive investigation. For the fetal deaths caused by complete congenital heart block associated with maternal autoantibodies, the evidence for a direct immunologic mechanism is now being established. As with isoimmunization, a more complete understanding of autoimmune-associated fetal death will open new avenues of management and therapy.
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PMID:Immunologic disease and fetal death. 311 69


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