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

Neonatal lupus erythematosus (NLE) syndrome is a result of the transfer of autoantibodies produced by the mother, across the placenta, to the fetus. NLE is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental SLE, induced by immunization with the monoclonal anti-DNA 16/6 Id, produce a variety of autoantibodies including anti-Ro and anti-La antibodies, we examined the relevance of NLE in the murine system. Offspring of SLE-afflicted BALB/c mothers possessed antibody titers to the 16/6 Id, ssDNA, and nuclear extract, which gradually declined until reduced to normal levels by day 60 after delivery. Antibody titers in the sera of the mothers remained elevated throughout this period. Electrocardiograms were recorded from groups of neonates from mothers with experimental SLE. The results indicated that a high percentage of the offspring had defects in their conduction system including first, second, and third degree heart block; significant bradycardia; and wide QRS complex. Normal patterns were observed in offspring of healthy mothers. Experiments done with mice that were exposed to SLE-related autoantibodies early in their development indicated that offspring to mothers with experimental SLE were neither protected nor more susceptible to disease induction by the 16/6 Id.
 ...
PMID:Neonatal lupus erythematosus in offspring of mothers with experimental systemic lupus erythematosus. 128 98

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)
 ...
PMID:Neonatal lupus syndromes. 128 97

Neonatal lupus erythematosus (NLE) is a well established subset of Lupus erythematous (LE). Three chinese female neonates presented to our Skin Centre, from May 1990 to May 1991 with NLE. All had skin lesions without congenital heart block and systemic problems. Two had photosensitive annular erythematous lesions on scalp, urticarial lesions on body, and one with facial atrophy, with aplasia cutis congenita. The biopsies were non specific while one had C3 in vessel walls. The major serogical marker was anti La antibody/SSB in two babies and anti Ro antibody/SSA in one. Two mothers were known cases of LE and one, was undiagnosed. Two infants were treated with short term low dose prednisolone. The infants will require long term follow up with paediatricians, in view of the fact that they can develop SLE later. The diagnosis of NLE is emphasised in infants with facial lesions to avoid delay in diagnosis, and full work up in both infants and mother is necessary.
 ...
PMID:Cutaneous neonatal lupus erythematosus in Chinese neonates. 130 64

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.
 ...
PMID:[Neonatal lupus erythematosus as an example of passively acquired autoimmunity]. 139 30

In patients with systemic lupus erythematosus in remission pregnancy has a good prognosis for mother and foetus. Pregnancy does not have a negative effect on systemic lupus erythematosus in remission, either in the short or the long term. Immuno-regulatory drugs should not be withdrawn before or after start of pregnancy, and flares during pregnancy can be treated with steroids and/or azathioprine. The risk of flare is very high in the puerperium, and can be prevented by steroids. All patients are at high risk of losing the foetus, particularly patients with antiphospholipid antibodies. Patients at high risk should be given low doses of aspirin (80 mg daily) from the first day of pregnancy. Neonatal lupus and congenital heart block are rare, and are associated with the presence of anti-SSA and anti-SSB antibodies in the maternal blood.
 ...
PMID:[Systemic lupus erythematosus and pregnancy]. 163 43

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.
 ...
PMID:[Neonatal lupus]. 236 16

The obstetrician is concerned with disseminated lupus because it is a relatively common illness which particularly affects women of reproductive age. This article reviews the literature in order to try to find an answer to the question: what effect pregnancy has on lupus and lupus on pregnancy. As far as the mother is concerned, pregnancy does not seem to increase the likelihood of relapse. Those women are most in danger who had renal disease before the pregnancy and still more if the renal lesions were active at the time of conception. There is no place any more for therapeutic termination of pregnancy nor probably for systematic treatment with cortisone. The prognosis for the fetus is more serious and does not depend on the maternal state. Fetal loss seems to be of the order of 40%, particularly due to non-specific complications of the illness. Neonatal lupus is linked to the presence of anti-Ro antibodies and to a particular HLA type. Finally, the criteria to be observed for looking after the mother and fetus are summarised.
 ...
PMID:[Systemic lupus and pregnancy. General review]. 268 68

Neonatal lupus erythematosus is a rare syndrome occurring in the first six months of life. It is characterized by the presence of Ro antibodies in the mother and affected infant, and at least one clinical manifestation--cardiac, hematologic, or dermatologic. If present, congenital heart block is irreversible and may be life-threatening in the neonatal period. Mothers of infants with neonatal lupus erythematosus may be asymptomatic when the affected infant is born but may later develop connective-tissue disease. The affected infants are at risk to develop systemic lupus erythematosus during adolescence or adulthood.
 ...
PMID:Neonatal lupus syndrome. 330 87

Neonatal lupus syndromes consist of transient cutaneous and hematologic abnormalities and permanent cardiac disorders all of which result from a common pathogenetic mechanism, the passive transfer of maternal autoantibodies. Detrimental antibodies such as SSA/Ro and SSB/La gain access to the fetal circulation via the normal active transport system of the trophoblast tissue which is operative after 20 weeks gestation. Despite functional maturation of the cardiac conduction system by 16 weeks gestation, fetal bradycardias are most often encountered after this time. Several lines of evidence are advanced in this review to support the role of myocarditis as the initial consequence of autoantibody attack on the fetal heart. The end result of this inflammatory insult is permanent fibrosis manifest as complete congenital heart block (CCHB). Despite the clearly demonstrated presence of SSA/Ro and SSB/La in fetal tissues as well as their fluctuation in quantity during the cell cycle, the precise accessibility of these antigens to their respective autoantibodies in unknown at the present time. However, ultraviolet light is reported to induce cell surface expression of SSA/Ro on cultured keratinocytes. The recognition of CCHB by fetal echocardiogram is presented. The rationale for the use of dexamethasone which crosses the placenta in an active form to treat fetal immune effector functions is discussed. Intense maternal plasmapheresis in an attempt to rapidly decrease maternal autoantibodies may provide another approach to the management of CCHB. Through increasing knowledge of this model of "passively acquired pure" systemic lupus erythematosus, insights into mechanisms of tissue injury and strategies for treatment will emerge.
 ...
PMID:Passively acquired autoimmunity and the maternal fetal dyad in systemic lupus erythematosus. 354 83

Fetal death occurs in about 1/3 of pregnancies in patients with systemic lupus erythematosus (SLE). It is largely predicted by lupus anticoagulant (estimated by activated partial thromboplastin time) and/or antibody to cardiolipin. These antibodies are not synonymous. Neonatal lupus appears in a minority of infants born to women with antibody to the Ro/La antigens. Hypocomplementemia is common in SLE pregnancies, as in pregnancy induced hypertension. Lupus exacerbation is uncommon either during or after pregnancy. Prematurity and fetal death are the greatest hazards.
 ...
PMID:Hazards of lupus pregnancy. 361 48


1 2 3 4 5 6 7 8 9 Next >>