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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 clinical manifestations of pediatric systemic lupus erythematosus (SLE) are similar to those seen in adults with SLE with increased frequency of the following features: hepatosplenomegaly, chorea, nephritis, and avascular necrosis. Similarly, pediatric SLE patients are now showing the same improvement in survival as adult SLE patients, and it is no longer felt that the course of childhood-onset SLE is more severe than that seen in adult-onset SLE. Children of mothers with SLE can develop both transient and persistent features of SLE in the neonatal period. Transient features include photosensitive discoid rash, cytopenia, hepatosplenomegaly, myocarditis, and pericarditis; the permanent features include congenital complete heart block, endomyocardial fibroelastosis, and other structural cardiac defects.
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PMID:Pediatric systemic lupus erythematosus and neonatal lupus. 815 96

Our study ellucidates the utility of endomyocardial biopsy (EMB) in various cardiac-muscle disorders seen in a tropical country like India. The procedure has been successfully performed in 501 patients (572 procedures) at our centre from April 1985 to December 1992. This included 60 infants and children. The indications were dilated cardiomyopathy (DCM) in 214, non-specific aortoarteritis in 91, rheumatic heart disease in 75, restrictive cardiomyopathy in 45, constrictive pericarditis in 14 and miscellaneous in 62 patients. There was no mortality, however, one patient developed cardiac tamponade and another sustained ventricular tachycardia requiring cardioversion. There was transient atrial fibrillation in six patients and all these had acute rheumatic heart disease. Transient complete heart block occurred in six patients with underlying left-bundle branch-block. Histological examination of EMB revealed myocarditis in 34/214 (15.4%) patients in DCM group and helped in following up these cases on immunosuppressive treatment. In the presence of restrictive haemodynamics it could identify amyloidosis in four patients. It was also helpful in differentiating between endomyocardial fibrosis and chronic constrictive pericarditis. In patients with non-specific aorto-arteritis significant histological changes of inflammatory myocarditis were observed in patients especially in congestive heart failure. Furthermore, it was helpful in identifying the nature of cardiac tumour in one patient. Its utility has also been evaluated in disorders, including rheumatic heart disease, peripartum cardiomyopathy and systemic disorders like systemic lupus erythematosis. Even in the absence of cardiac-transplant programmes at national level we have found EMB to be a useful investigation in a tropical country like India.
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PMID:Endomyocardial biopsy--technical aspects experience and current status. An Indian perspective. 818 91

The aim of the study was to determine if autoantibodies have distinctive qualitative or quantitative characteristics in children with congenital heart rhythm disorders compared to unaffected infants. Sera of fifty-three infants with different heart rhythm disorders and eight unaffected children born to SLE mothers were tested for antibodies to Ro(SS-A), La(SS-B) and U1RNP (including recombinant A-, C-, 68 kD-proteins) using enzyme immunoassays and immunoblot. 21 sera from affected and 8 sera from healthy children reacted in counter-immunoelectrophoresis with Ro(SS-A). 8/8 infants with third degree atrioventricular (AV)-block, 4/8 with first degree AV-block, one with sinusbradyarrhythmia (SB) and another with SB/first degree AV-block were anti-52 kD Ro(SS-A) positive compared to 7/8 healthy infants. Infants with third degree AV-block had significantly higher anti-52 kD Ro(SS-A) levels than those with the milder heart rhythm disorders (P < 0.01) and the healthy group (P < 0.002). Anti-La(SS-B) antibodies were detected significantly (P < 0.01) and in significantly higher titres (P < 0.01) in AV III than in other types of congenital heart block. Anti-U1RNP-68 kD antibodies were positive in 3/8 healthy, 2/8 first degree AV-block and in 1/8 third degree AV-block cases. Third degree AV-block was more frequently found in female infants (P < 0.05). It was concluded that the influence of antibody levels to the 52 kD Ro(SS-A) and La(SS-B) components and the association of anti-52 kD Ro(SS-A), La(SS-B) and -60 kD Ro(SS-A) antibodies, as well as the sex, on the severity of congenital AV-conduction defects suggests involvement of these factors in the pathogenesis of these disorders.
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PMID:Detection of autoantibodies to Ro(SS-A), La(SS-B) and U1RNP in different congenital heart rhythm disorders using immunoblot and enzyme immunoassay. 819 4

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by complete congenital heart block and/or transient skin lesions of subacute cutaneous lupus erythematosus. We report that in approximately 10% of cases of NLE with heart block or skin disease, liver disease also occurs (4 of 35 cases in our series). Cholestasis was the major feature in our cases. Although the cholestasis may be severe, the disease process appears to be transient and surviving babies have been healthy on follow-up. In one liver examined for antibody deposition, IgG antibody deposits, presumably of maternal origin, were present. Three maternal sera were examined for autoantibodies, including liver-specific autoantibodies. No liver-specific autoantibodies were found. Rather, the maternal autoantibodies too were the ubiquitous Ro/SSA-associated autoantigens. The autoantibodies bound the 60 kDa SSA/Ro ribonuclear protein (three of three sera), the 52 kDa SSA/Ro protein (two of three sera) and the SSB/La ribonuclear protein (two of three sera).
Lupus 1993 Oct
PMID:Neonatal lupus liver disease. 830 29

A critically ill infant with congenital complete heart block and neonatal lupus was treated with pulse steroids, exchange transfusion and intravenous gammaglobulin. Transient improvement in clinical status and laboratory results occurred, although the infant died. Based on this report and review of prior experience with immunosuppressive therapy, prenatal treatment of the mother with betamethasone or dexamethasone has been successful in resolving the cardiomyopathy/myocarditis associated with neonatal lupus. Postnatal treatment of the infant with steroids appears to be beneficial for persistent hepatic and hematologic manifestations. The evidence for exchange transfusion is less certain.
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PMID:Treatment of neonatal lupus: case report and review of the literature. 837 Nov 99

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.
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PMID:Atrioventricular conduction in children of women with systemic lupus erythematosus. 842 Feb 43

Human adult cells are protected from complement-induced damage in part by membrane cofactor protein (MCP, CD46). To examine fetal characteristics which might influence autoantibody-mediated diseases acquired in utero, such as heart block in neonatal lupus, the tissue expression of MCP was studied. Using a high ratio of acrylamide:bisacrylamide, immunoblots of tissues from six fetuses (aged 19-24 weeks) probed with rabbit anti-MCP antibodies revealed a band at 60 KD in addition to the known 65 KD and 55 KD isoforms which comprise the codominant allelic system of MCP. Five fetuses expressed the most common MCP polymorphism (predominance of the 65 KD isoform, upper band alpha-phenotype) in the kidney, spleen, liver and lung. In contrast, all hearts from these five fetuses demonstrated a different pattern in which there was a marked decrease in the intensity of the 65 KD band and accentuation of the lower molecular weight bands. In a sixth fetus, which expressed the second most common polymorphism (equal expression of the 65 KD and 55 KD MCP isoforms, alpha beta-phenotype), the heart was similar to the other tissues. These studies confirm the expression of MCP in early gestational life. Preferential expression of the MCP beta-isoform in the majority of fetal hearts irrespective of the phenotype of other organs, suggests tissue-specific RNA splicing or post-translational modification which may relate to autoantibody-mediated injury in diseases such as neonatal lupus.
Lupus 1995 Aug
PMID:Ontogeny of membrane cofactor protein: phenotypic divergence in the fetal heart. 852 26

Antibodies to Ro(SS-A) are an important laboratory parameter of congenital heart block (CHB), but the maternal presence of anti-Ro(SS-A) antibodies does not always lead to this fetal acquired autoimmune disease. The current study investigated quantitative and qualitative differences of anti-52, -60 kD Ro(SS-A), 0La(SS-B) and -U1RNP(-C, -A, -68 kD) autoantibodies (either IgG or IgM) in sera derived from 16 infants with CHB and their mothers compared to eight healthy anti-Ro(SS-A) positive infants (controls) born to SLE mothers. No serum sample contained IgM auto-antibodies of the specificities investigated. Anti-60, 52 kD Ro(SS-A) and La(SS-B) IgG antibodies coincided in 9/16 CHB cases, exclusively (P < 0.05). Associated anti-52 kD Ro(SS-A) and -La(SS-B) IgG antibodies were detected in 14/16 CHB and in 3/8 control cases (P < 0.05). Anti-U1RNP 68 kD antibodies occurred in 1/16 CHB infants and in 4/8 controls. In general, newborn and maternal antibody patterns and reactivities were similar in all cases investigated. The fetal CHB sera contained significantly higher IgG levels of anti-52 kD Ro(SS-A) (P < 0.005) and -La(S-B) (P < 0.015) compared to control samples. Sera from mothers with CHB children had significantly higher levels of anti-52 kD Ro(SS-A) (P < 0.015) and -La(SS-B) (P < 0.015) IgG antibodies than those of the control group. Thus, the coincidence of anti-52 kD, -60 kD Ro(SS-A) and -La(SS-B) IgG antibodies as well as significantly increased levels of antibodies to 52 kD Ro(SS-A) and La(SS-B) are associated with evidence of complete congenital heart block. The data suggest that the known associated humoral autoimmune findings are exclusively of maternal origin.
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PMID:Significantly increased maternal and fetal IgG autoantibody levels to 52 kD Ro (SS-A) and La(SS-B) in complete congenital heart block. 857 23

Neonatal lupus is strongly associated with antibodies reactive with SSA/Ro-SSB/La proteins, independent of maternal disease activity or classification. We sought to determine whether the fine specificity of antibody profiles remains stable or evolves over time and whether these findings relate to clinical status. Sera from 23 mothers whose children had neonatal lupus (22 heart block, one skin) were evaluated by SDS-immunoblot. For each mother two samples were available at least 13 months apart; the mean duration of time between testing was 45 months +/- 27 S.D. (range 13-108 months). Twenty-two of the 23 initial profiles were identical to the results obtained in a later sample. The health status of seven (30%) of 23 mothers changed after the birth of the affected infant but the immunoblot specificity of the antibodies remained unchanged. SLE was the initial and final diagnosis in the only mother whose profiles differed, with development of weak reactivity to 48 kD SSB/La in addition to the 52kD SSA/Ro after 14 months. In conclusion, the fine specificity of anti-SSA/Ro-SSB/La antibodies as assessed by immunoblot is highly stable for years. Progression of clinical status was not associated with a concomitant change in antibody profile.
Lupus 1996 Jun
PMID:Stability of immunoblot profile of anti-SSA/Ro-SSB/La antibodies over time in mothers whose children have neonatal lupus. 880 92

Central nervous system involvement in neonatal lupus erythematosus (NLE) has not been previously reported. We report four patients with NLE, all with complete congenital heart block and three with cerebral ultrasound and color Doppler flow imaging (CDFI) studies demonstrating evidence of associated vasculopathy in the gangliothalamic vasculature. CDFI confirmed blood flow through the affected vessels, indicating that blood flow was not compromised at this early stage. Short-term follow-up revealed no signs of progression of the vasculopathy, focal ischemia, gangliothalamic atrophy, or neurological impairment. Nevertheless, the implications of this finding with respect to the natural history of NLE remain to be defined, particularly in cases in which the disease develops into systemic lupus erythematosus later in life. Besides specific diagnostic studies for NLE, cerebral ultrasound, and CDFI studies are mandatory in all cases of complete congenital heart block, regardless of whether mothers are diagnosed as having connective-tissue disease or not. Neonates with signs of vasculopathy in the gangliothalamic region should be examined for NLE.
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PMID:Central nervous system vasculopathy in neonatal lupus erythematosus. 888 46


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