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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neonatal lupus syndrome is considered a model of passively acquired autoimmune disease. The first 10 newborns born to mothers with connective tissue disease and positive for anti-SSA/Ro antibodies enrolled in a follow-up program to evaluate the incidence of cardiac, hepatobiliary, hematologic, echoencephalographic, and cutaneous manifestations until 9 months of age are described in this study. No congenital heart block was observed, but only transient rhythm alterations were observed. In all, 1 infant showed typical neonatal lupus syndrome skin lesions at 3 months of age. During the neonatal period, echoencephalographic alterations were found more frequently, whereas at follow-up, hepatic and hematologic alterations were more often observed. In all, 1 baby showed persistent neutropenia. A standard program that enrolls all infants born to mothers with anti-SSA/Ro autoantibodies, who are at risk of developing neonatal lupus syndrome, should also include tests performed some time after birth, as a number of clinical manifestations might appear at a late stage.
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PMID:Infants born to mothers with anti-SSA/Ro autoantibodies: neonatal outcome and follow-up. 1805 59

Neonatal lupus is a rare disease, with a prevalence of 1:17,000-20,000 child births. The disease is attributed to autoantibodies such as anti-Ro and anti-La. These antibodies are a common finding in Systemic Lupus Erythematosus and Sjogren disease patients. The clinical manifestations include dermatological, hematological, hepatic, neurological and cardiac involvement. Cardiac disease may be presented as atrioventricular conduction block, cardiomyopathy or endocardial fibroelastosis. If the fetus's heart is affected, the death rates may be as high as thirty percent, and dilated cardiomyopathy may evolve. About 60% of infants will require a permanent pacemaker. Routine screening and early detection are crucial for the initiation of effective treatment. Nevertheless, preventive treatment for all mothers is not recommended due to the low prevalence of the disorder and the common side effects. This article reviews the current knowledge on pathophysiology, and treatment options in neonatal lupus erythematosus.
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PMID:[Neonatal lupus erythematosus--cardiac manifestations and atrio-ventricular conduction block]. 1825 46

Neonatal lupus erythematosus is an uncommon transplacentally acquired autoimmune disorder. The most common clinical manifestations are skin rash, congenital atrioventricular block, thrombocytopenia, leukopenia, anemia, and hepatosplenomegaly. Usually, the skin rash resembles subacute cutaneous lupus, but different forms of rash have been reported in neonatal lupus erythematosus and some are rare forms. NLE should be suspected in babies with atypical skin lesions, even if present at birth.
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PMID:Neonatal lupus erythematosus: an acquired autoimmune disorder and its cutaneous manifestations. 1866 51

Neonatal lupus is an uncommon condition associated with maternal anti-Ro autoantibodies. Findings may include cutaneous lupus lesions, third-degree heart block, cardiomyopathy, hepatobiliary disease, and/or thrombocytopenia or other hematologic cytopenias. It is common for only one organ to be affected, but any combination of organ involvement may occur. Recent studies have raised the possibility that the central nervous system may also be affected, but if it is, it is generally apparently asymptomatic. The most common severe manifestation of neonatal lupus is third-degree heart block, which usually begins during the second trimester of gestation. Attempts have been made to prevent the development of heart block, most often by treating the mother with systemic corticosteroids during pregnancy. There is not yet consensus as to the value of intervention during pregnancy. The neonatal lupus disease process is transient, although third-degree heart block, once established, is permanent. Cutaneous lesions tend to resolve completely and affected individuals tend to be healthy later in childhood. There does appear to be an increased risk for children who have had neonatal lupus to develop autoimmune diseases later in childhood or adulthood. The magnitude of that risk is uncertain. Mothers, who are often asymptomatic at the time of delivery of a baby with neonatal lupus, tend eventually to develop signs and symptoms of autoimmune disease.
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PMID:The clinical spectrum of neonatal lupus. 1879 91

Lupus erythematosus is a chronic and inflammatory multiorgan disease with variable clinical appearance and variable course. Most patients with systemic lupus erythematosus show cutaneous manifestations and conversely, all forms of cutaneous LE may change into a systemic involvement. Specific lesions of cutaneous LE are classified in different subtypes of acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CDLE) and intermittent cutaneous lupus erythematosus (ICLE) according to clinical, histological and immunoserological parameters. Regular laboratory tests are important to monitor the activity and course of the disease or side effects of the therapy. In case of clinical or laboratory dysfunctions of internal organs, additional technical investigations are necessary. Histology is needed to support clinical diagnosis. A large number of drugs are able to induce SCLE, e.g. hydrochlorothiazide, terbinafine, or angiotensin-converting enzyme inhibitors. Drug-induced SCLE can be differentiated by possible complementary immunoserological parameters. Neonatal lupus can be induced by transplacental transmission of maternal anti-Ro(SS-A) and anti-La(SS-B)-antibodies. Children with neonatal lupus might suffer from congenital atrioventricular block. Their mothers may suffer from active LE, but can be clinically healthy as well. As a consequence, pregnancies at risk should be monitored in short intervals by serial echocardiographic interventions. Protection against UV light is recommended for all types of CLE. There are some topical and many systemic treatment options e.g. topical and systemic glucocorticosteroids, antimalarial drugs, dapsone, azathioprine, or mycophenolate mofetil with different response to skin or organ involvement.
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PMID:The different faces of cutaneous lupus erythematosus. 1935 21

Neonatal lupus erythematosus (NLE) develops in foetuses of mothers with Ro/SSA and La/SSB antibodies and may include foetal atrioventricular block and dermatologic manifestations. In this study, we investigated postnatal Ro and La IgG, IgA and IgM antibody levels up to 1 year of age in 32 children born to Ro/SSA positive mothers. Antibody levels were correlated with NLE manifestations, and the role of breast feeding in transfer of autoantibodies from mother to child was evaluated. Ro52, Ro60 and La IgG antibodies all transferred from the mothers to their foetus in utero and were present in the infant at birth as detected by enzyme-linked immunosorbent assay using recombinant antigens and a synthetic peptide. A significant decrease in Ro52, Ro60 and La IgG autoantibody levels of the infants was observed from birth to 4-5 weeks of age (P < 0.05, P < 0.05 and P < 0.01). Ro- and La-specific IgA and IgM antibodies were detected in the serum from a subset of mothers. However, Ro- and La-specific IgA and IgM antibody levels were low or non-detectable in children raised both with and without breastfeeding. Furthermore, NLE skin lesions developed independently of breastfeeding. Our findings support a role for placental materno-foetal transfer of IgG autoantibodies in the pathogenesis of NLE and indicate that refraining from breastfeeding does not protect from NLE skin involvement.
Lupus 2009 Aug
PMID:Serologic follow-up of children born to mothers with Ro/SSA autoantibodies. 1957 3

Neonatal lupus is a model of passively acquired autoimmunity whereby anti-SSA/Ro-SSB/La antibodies target the fetal heart and neonatal skin in a minority of cases. Since neuro-psychiatric impairment has been reported in humans and mice exposed prenatally to a variety of maternal autoantibodies including anti-Ro/La, this study was initiated to evaluate the potential neurotoxic effects of these specific autoantibodies and the overall frequency of autoimmune diseases, general health, and somatic growth of children with neonatal lupus and their unaffected siblings. In addition to the general health questionnaires maintained on family members enrolled in the Research Registry for Neonatal Lupus (RRNL), specific questionnaires related to neuro-psychiatric development were sent to all mothers whose children (both affected and unaffected) were older than 5 years of age. Controls consisted of healthy friends. Of 121 anti-Ro exposed children meeting the inclusion criteria, information was returned on 104 (33 cardiac manifestations of neonatal lupus, 20 rash, and 51 unaffected siblings) and 22 of the friend controls. The mean age of all of the children was 14.5 years (range 5-39). In total, 42 (40%) of the 104 anti-Ro exposed children were reported to have a neuro-psychiatric disorder, compared with 6 (27%) of the friend controls (p = 0.34). For 8 (24%) of the congenital heart block (CHB) children (6 boys, 2 girls) the mothers reported attention problems. Four, all boys, were on stimulants. Of the rash children, 4 (20%) (2 boys, 2 girls) had attention problems with one boy on Ritalin. Of the unaffected siblings, 9 (18%) (8 boys and 1 girl) had attention problems with 3 boys on stimulants. One (5%) of the control children (a girl) had attention problems, not requiring therapy. There was no statistical difference in attention problems between the groups (p = 0.120). Behavioral problems were present in all groups with no statistical differences noted. The prevalence of depression, anxiety, developmental delays, learning, hearing, and speech problems were not significantly different between groups. In the CHB children, one boy has nephrotic syndrome and one girl has psoriasis. In the rash children, one girl has juvenile rheumatoid arthritis. In the unaffected group there are five children with autoimmune diseases, two with inflammatory bowel diseases (one boy and one girl), one boy has a spondyloarthropathy, one girl has alopecia areata and one young woman has Antiphospholipid syndrome. In the control group one boy has Henoch Schonlein purpura. There were four cases of hypothyroidism, possibly secondary to Hashimoto's thyroiditis, three in boys with CHB and one in a girl with rash. None of the unaffected siblings or controls had hypothyroidism. Parental reporting of neuro-psychiatric abnormalities was high in anti-Ro exposed children regardless of the neonatal lupus manifestation. However, medication use was limited and although the frequency of this reporting was greater than friend controls, it did not reach significance.
Lupus 2010 Mar
PMID:Frequency of neuro-psychiatric dysfunction in anti-SSA/SSB exposed children with and without neonatal lupus. 2000 45

Neonatal lupus erythematosus is an uncommon disease caused by transplacental passage of maternal anti-Ro (SS-A), anti-LA (SS-B), or anti-U1RNP antibodies. Cutaneous findings of neonatal lupus are variable, but annular, erythematous plaques occurring within a few weeks of birth are most typical. Cutaneous lesions of congenital onset lupus erythematosus can differ from that of neonatal lupus erythematosus, presenting with atrophy or scarring, and less commonly, erosions. We report an unusual case of congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease.
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PMID:Congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease. 2019 33

Neonatal lupus erythematosus is an immune-mediated disease caused by transplacental passage of maternal autoantibodies, primarily anti-Ro (SSA) and anti-La (SSB). The major clinical manifestations are congenital heart block, cutaneous lupus lesions, and hematologic problems. Hepatic, pulmonary, and neurological involvements are rare. We report a 5-day-old male neonate, born to a clinically asymptomatic mother, presenting with conjugated hyperbilirubinemia, cutaneous lupus lesions, congenital heart block, and thrombocytopenia. Both the neonate and his mother had high titers of antinuclear antibodies (1:640), anti-Ro (SSA), and anti-La (SSB) antibodies. The thrombocytopenia improved with prednisolone (2 mg/kg/day) for 14 days. The skin lupus rashes and bilirubin resolved 2 months later, and liver enzymes were completely normal by 6 months.
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PMID:Early cholestasis in neonatal lupus erythematosus. 2162 63

Neonatal lupus erythematosus is an uncommon maternal auto-antibody-associated disease characterized by cutaneous, cardiac, hepatic, hematological, neurological, and pulmonary involvement. A retrospective study was performed to review clinical manifestations, investigation results, outcomes of neonatal lupus erythematosus patients and their mothers at the Department of Pediatrics, Siriraj Hospital during 1993 to 2008. Seventeen neonatal lupus erythematosus patients (10 girls and seven boys) were identified. Cutaneous, cardiac, hepatobiliary, and hematological involvement was found in 70.6%, 64.7%, 52.9%, and 35.3% of infants, respectively. Skin lesions were erythematous patches (91.7%), subacute cutaneous lupus erythematosus (50%), petechiae (41.7%), persistent cutis marmorata (16.7%), and discoid lesions (8.3%). Congenital heart block was found in nine cases, and structural abnormalities were found in nine cases. All sera of patients were positive for antinuclear antibodies. Patients (87.5%) showed positive antiRo/SSA, and 50% had positive antiLa/SSB antibodies. Most neonatal lupus erythematosus mothers (64.7%) were asymptomatic. Five mothers were diagnosed with systemic lupus erythematosus, and one mother was diagnosed with mixed connective tissue disease. All maternal sera was positive for antinuclear antibodies and antiRo/SSA antibody. Seven patients required pacemaker implantation. The mortality rate was 11.8%, caused by congestive heart failure and pneumonia. Antinuclear antibody tests should be used as one of the screening tests in mothers or patients suspected of having neonatal lupus erythematosus.
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PMID:Neonatal lupus erythematosus: clinical character, investigation, and outcome. 2136 29


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