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)

The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.
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PMID:Neonatal lupus erythematosus. 51 88

A 22-year-old woman with systemic lupus erythematosus complicated by mild renal insufficiency and severe systemic hypertension inadvertently received an excessive amount of clonidine hydrochloride. In association with a presumed toxic level of clonidine in the serum, the patient developed abnormalities of cardiac conduction, including 2:1 atrioventricular block, complete heart block, 3:2 Wenckebach block, and first-degree atrioventricular block. The transient nature of these abnormalities, with the return of normal conduction upon the cessation of therapy with clonidine, implicates this drug as being capable of producing high-grade atrioventricular block at toxic levels.
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PMID:Cardiac toxicity of clonidine. 92 17

From January, 1979 to December, 1990, 105 pregnancies of the 87 mothers with systemic lupus erythematosus (SLE) were studied. There were 15 (14.29%) fetal losses. Among the 90 livebirths, 23 (25.5%) were moderately premature; and 1 (1.1%), was extremely premature. All but 2 (2.2%) had an Apgar score more than 7 at 1 minute. There was no neonatal death. Significantly lower birth body weights were noted compared with the matched control group (p = 0.0001). Birth body length and head circumference were not different. Only 1 of the 13 newborns who had been small for gestational age at birth had body weight and length less than the 3rd percentile during follow-up. Three (3.3%) newborns presented as congenital complete atrioventricular block (CCAVB). Their mothers were all positive for SSA (Ro) antibody. One of them obligatorily needed pacemaker implantation. ECG abnormalities including multiple ventricular premature contractions, wandering atrial pacemaker, sinus arrhythmia, and first degree A-V block were detected in another six newborns. Congenital cardiac defect with secundum type atrial septal defect was noted in two newborns (2.2%). Among the 59 mothers who had been tested for SSA antibody, 29 (49.1%) were positive. The incidence of complete A-V block was significantly higher in newborns of mothers with SSA antibody (p < 0.001). On the contrary, the frequency of fetal loss has higher in newborns of mothers without SSA antibody (p = 0.0043).
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PMID:Newborns of Chinese mother with systemic lupus erythematosus (SLE). 129 44

Neonatal lupus erythematosus is a typical example of passively acquired autoimmune disease. Congenital heart block is the main complication caused by transplacentally transferred maternal IgG antibodies directed against SS-A- or SS-B-antigen. While the transient lupus dermatitis occurs post partum and is often triggered by UV-light exposition, the irreversible cardial damage takes place between the 18. and 24, week of pregnancy. During this period 52-kD-SS-A- and 48-kD-SS-B-antigen are expressed in the fetal cardial tissue. The autoimmune reaction leads to an irreversible fibrotic destruction of the atrioventricular node. The resulting AV block and an antibody-induced perimyocarditis support the development of fetal bradyarrhythmia and hydrops. The very early diagnosis of a fetal heart block in bradycardiac fetus by echocardiography is essential for a consecutive therapy with steroids. By a reduction of the maternal antibodies an improvement of cardial symptoms in the fetus may be achieved in cases with fetal hydrops. Pregnant women at high risk with known connective tissue disease, previous children with congenital heart block, high titers of SS-A-/SS-B-antibodies and immunogenetic predisposition (HLA-DR3) have to be monitored intensively because of the possibility of a prophylaxis with dexamethasone and, in some selected cases, plasmapheresis.
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PMID:[Neonatal lupus erythematosus as an example of passively acquired autoimmunity]. 139 30

We describe 2 patients with systemic lupus erythematosus (SLE) and high grade atrioventricular (AV) block. In one patient anti-Ro antibodies were positive. This antibody has been associated with congenital complete heart block in children of mothers with SLE; however, its role in adult high grade AV block is not clear. We reviewed the 8 published cases of adults with SLE and high grade AV block. The possible mechanisms that could be implicated in the pathology of high grade AV block are discussed.
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PMID:High grade atrioventricular heart block in 2 adults with systemic lupus erythematosus. 179 34

The majority of anti-nuclear antibodies do not have a proven pathogenic role in systemic lupus erythematosus (SLE). The pathogenicity of native anti-DNA antibodies, suggested by clinical data, is difficult to confirm. It is linked to several factors: isotype and avidity of these antibodies, affinity for DNA, size of immune complexes. DNA, anti-DNA antibodies and cross-reactive anti-DNA idiotypes (16/6) have been isolated from human and NZB mouse glomeruli. Moreover, DNA has a particular affinity for the glomerular membrane due to its cross-reactivity with some constituents of this membrane. Anti-SSA (Ro) antibodies may play a role in some cases of nephropathy in SLE and participate in photosensitization. They are associated with neonatal lupus, with some cases of fetal death in black women and with the atrioventricular heart block. However, factors other than these antibodies are needed to induce such lesions. Anti-U1 RNP antibodies do not protect against kidney involvement in SLE; however, they can decrease suppressive function by penetrating into suppressor T cells. Anti-Sm antibodies may have particular immunoregulation properties. Some antinuclear antibodies may at least in part, be responsible for the lesions in SLE, in conjunction with other still-unknown factors.
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PMID:[Pathogenic role of antinuclear antibodies in lupus disease]. 219 47

Even in 1990 there are still conflicting views concerning oral contraception (OCs) and risks associated with pregnancy in women with systemic lupus erythematosus. With regard to OCs, all authors agree that estrogen-progestin combinations are harmful, but the best alternative hormonal contraception has yet to be found. Progestin-based micropills seem to be harmless; cyproterone acetate appears to have no side effects and its usefulness in preventing recurrences is being evaluated. With regard to pregnancy, it would be wise not to contemplate having a child until 6 months have passes since onset of remission. Blood pressure, platelet count, and serum creatinine and uric acid levels must be closely monitored. 2 types of antibodies may be present in the mother and may be responsible for fetal complications. These are antibodies to phospholipids (antiprothrombinase, anticardiolipin, antibodies responsible for dissociated treponema serology), which expose patients to spontaneous abortion or intrauterine death, and the antibody to SS-A (or anti-Ro), which exposes patients to fetal cardiomyopathy and congenital atrioventricular block. (author's modified)
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PMID:[Contraception and pregnancy in systemic lupus erythematosus]. 223 88

Neonatal lupus erythematosus is characterized by congenital atrioventricular heart block and/or transient lupus skin lesions frequently similar to those seen in patients with subacute cutaneous lupus erythematosus. The mothers have anti-SSA (Ro) and/or anti-SSB (La) antibodies. The syndrome is important to recognize because newborns may develop complete heart block with congestive heart failure. However, in the majority of cases, neonatal lupus erythematosus is a relatively benign disorder and cutaneous lesions generally disappear completely by 6 months of age.
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PMID:[Neonatal lupus]. 236 16

In view of the association of congenital heart block with maternal antibody to cellular antigen Ro (SSA), and one report linking anti-Ro with myocarditis in a patient with myositis an association between anti-Ro antibodies and cardiac disease was sought in adults with systemic lupus erythematosus (SLE). Among 67 patients with SLE, of whom 36 were anti-Ro positive, a significantly higher prevalence of myocarditis and conduction defects was found in the anti-Ro positive group (eight of 36) than in those who were anti-Ro negative (one of 31) and healthy controls (one of 50). Of the 36 anti-Ro positive patients with SLE, three had symptoms diagnostic of myocarditis, and an electrocardiogram showed first degree atrioventricular block and unifascicular block in three cases (including one with myocarditis), right bundle branch block alone (two cases), and first degree atrioventricular block alone (one case). Complete atrioventricular block was not seen. In the anti-Ro negative group there was no myocarditis and only one case of conduction defect (right bundle branch block). Among healthy controls only one of 50 had first degree atrioventricular block. It is concluded that myocarditis and conduction defects are reasonably common in adults with SLE and are associated with anti-Ro antibodies.
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PMID:Possible association between anti-Ro antibodies and myocarditis or cardiac conduction defects in adults with systemic lupus erythematosus. 239 70

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


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