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Earl D Silverman - One of the best experts on this subject based on the ideXlab platform.
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ethnicity and Neonatal Lupus Erythematosus manifestations risk in a large multi ethnic cohort
The Journal of Rheumatology, 2021Co-Authors: Talia Diaz, Earl D Silverman, Edgar Jaeggi, Daniela Dominguez, Andrea M Knight, Carl A Laskin, Franklin Silverio, Linda T HirakiAbstract:Objective To evaluate the association between ethnicity and Neonatal Lupus Erythematosus (NLE), as well as specific NLE manifestations in a large multiethnic population. Methods We conducted a cohort study of the children (≤ 1 yr of age) seen in the NLE clinic at The Hospital for Sick Children (SickKids), between January 2011 and April 2019. The cohort was divided into European, non-European, and mixed European–non-European groups according to parent-reported child9s ethnicity (Canada Census categories). Outcomes were NLE and specific NLE manifestations (cardiac, cutaneous, cytopenias, transaminitis, and macrocephaly). The frequency of NLE and specific manifestations were compared between ethnic groups (Fisher exact test). We tested the association between ethnicity and (1) NLE risk, and (2) specific NLE manifestations with logistic regression models, including covariates for child9s sex, maternal rheumatic disease status during pregnancy, and maternal use of antimalarials during pregnancy (multiple comparisons threshold P Results We included 324 children born to 270 anti-Ro antibody–positive mothers. Median age at first visit was 1.8 (IQR 1.4–2.3) months, and median follow-up time was 12 (IQR 2–24) months. The majority was non-European (48%), with 34% European, and 18% mixed European–non-European. There was no significant association between non-European ethnicity (OR 1.18, 95% CI 0.71–1.94, P = 0.51), mixed European–non-European ethnicity (OR 1.13, 95% CI 0.59–2.16, P = 0.70), and NLE risk compared with European ethnicity. We also did not find an association between ethnicity and specific NLE manifestations in univariate or multivariable-adjusted models. Conclusion In a large multiethnic cohort, there was no association between a child9s ethnicity and NLE risk or specific NLE manifestations.
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non cardiac manifestations of Neonatal Lupus Erythematosus
Scandinavian Journal of Immunology, 2010Co-Authors: Earl D Silverman, Edgar JaeggiAbstract:Neonatal Lupus Erythematosus (NLE) is characterized by the transplacental passage of maternal anti-Ro and/or anti-La antibodies and characteristic illnesses in the foetus/neonate. Most attention has focused on the most serious complication- cardiac involvement. This article will focus on non-cardiac involvement. Skin involvement (cutaneous NLE) is present in 15-25% of children with NLE. The rash of NLE tends to be photosensitive but may be present at birth or in non-sun exposed areas. It is most frequently seen around the eyes, not in the malar area, but also occurs in other parts of the body. The pathology resembles the rash of subacute cutaneous Lupus Erythematosus. Anti-Ro antibodies are present in >95% with the remaining mothers having anti-U1RNP antibodies only. Asymptomatic elevation of liver function tests, which may be associated with evidence of cholestasis, is seen in 10-25% of cases of NLE. Mild hepatomegaly and less commonly splenomegaly may be present. Liver involvement seen in isolation or associated with other features. The pathology resembles idiopathic Neonatal giant cell hepatitis. Any haematological lineage, neutropenia and thrombocytopenia most commonly, may be affected by NLE. Haematological involvement is almost always asymptomatic. There are protean manifestations of neurologic involvement in NLE: hydrocephalus, non-specific white matter changes, calcification of the basal ganglia and a 'vasculopathy'. The most unusual feature of NLE is the radiographic finding of stippling of the epiphyses (chondrodysplasia punctata). Overall, non-cardiac involvement of NLE is more common than cardiac. The study of these manifestations may lead to new insight into how autoantibodies lead to disease.
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the importance of the level of maternal anti ro ssa antibodies as a prognostic marker of the development of cardiac Neonatal Lupus Erythematosus a prospective study of 186 antibody exposed fetuses and infants
Journal of the American College of Cardiology, 2010Co-Authors: Edgar Jaeggi, Carl A Laskin, Robert J Hamilton, John Kingdom, Earl D SilvermanAbstract:Objectives The purpose of this study was to determine whether cardiac complications of Neonatal Lupus Erythematosus (NLE) are related to maternal anti-Ro and anti-La autoantibody-levels. Background Autoantibody-positive mothers are frequently referred for serial echocardiography because of the elevated fetal risk of developing immune-mediated heart block. Little is known why only some and not all offspring are affected. Methods All cases referred since 2000 for serial fetal echocardiography or cardiac complications related to maternal antibodies were included. Patients without cardiac NLE (group 1) and with cardiac NLE (group 2) were compared. Antibody levels were measured by enzyme-linked immunosorbent assay with a cutoff value of 8 U/ml for a positive test result. Results Group 1 included 146 serially screened fetuses with normal pregnancy outcomes. Group 2 consisted of 40 fetuses/neonates with a diagnosis of heart block or endocardial fibroelastosis or both, and included 4 fetuses diagnosed during serial screening. All cardiac complications were associated with moderate (≥50 U/ml; 15%) or high (≥100 U/ml; 85%) maternal anti-Ro levels, independently of anti-La antibody titres. The event rate of complete heart block was 5% for prospectively screened fetuses with Ro-values ≥50 U/ml (odds ratio: 7.8) and 0% for fetuses with lower titres (p Conclusions Our findings support that the amount of maternal antibodies, rather than their presence, is associated with fetal tissue injury. As anti-Ro levels correlate with the risk of cardiac complications, serial echocardiography should be limited to women with high anti-Ro-titres.
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autoantibody response to the ro la particle may predict outcome in Neonatal Lupus Erythematosus
Clinical and Experimental Immunology, 2008Co-Authors: Earl D Silverman, Ronald M Laxer, Jill P Buyon, Richard Hamilton, P Bini, J L Chu, Keith B ElkonAbstract:This study was undertaken to determine the role of antibodies against both recombinant Ro (r-Ro) and La (r-La) proteins and polypeptides derived from the recombinant La protein in predicting fetal and Neonatal outcome in children at risk to develop Neonatal Lupus Erythematosus (NLE). All sera were obtained in the perinatal period and quantitative ELISA assays were used. We collected 41 maternal sera within 2 months of delivery of a child with NLE (21 with congenital heart disease block (CHB) and 20 with dermatologic NLE) and 19 sera from anti-Ro and/or anti-La antibody-positive mothers with systemic Lupus Erythematosus (SLE) who delivered a child without NLE. All sera were tested for anti-r-La and anti-r-Ro antibodies by ELISA, and most sera were tested for antibodies directed against La polypeptides by immunoblot. We found significantly higher anti-r-La antibody levels in the sera from mothers of children with NLE compared with sera from mothers of unaffected children (0.67 +/- 0.43 versus 0.14 +/- 0.30; P < 0.0001). There was a statistically significant difference in the mean anti-r-La levels between the sera of mothers of children with CHB compared with dermatologic NLE (0.51 +/- 0.45 versus 0.83 +/- 0.37 respectively; P = 0.0091). When we examined antibodies directed against the recombinant 52-kD Ro protein, there was a statistically significant elevation of titres in the sera of mothers of NLE children (0.77 +/- 0.35) compared with non-NLE mothers (0.29 +/- 0.39; P < 0.0001). There was no difference in the r-Ro levels between mothers of children with dermatologic NLE compared with CHB (0.82 +/- 0.37 versus 0.71 +/- 0.74; P = 0.32). When we examined polypeptides derived from the recombinant La protein, the mean number of polypeptides recognized by sera from mothers of children with NLE was significantly higher than the mean number of polypeptides recognized by sera from mothers of unaffected children (5.1 +/- 0.54 versus 2.3 +/- 0.54 respectively; P < 0.001). More importantly, when we examined the individual polypeptides, we found that only sera from mothers of children with NLE and not from mothers of unaffected children recognized a polypeptide designated DD (30% versus 0%, respectively). These studies indicate that the autoantibody response to the Ro/La particle can differentiate sera from mothers of children with NLE and sera from mothers of unaffected children. Furthermore, there was a difference in the anti-La autoantibody response between mothers of children with CHB and dermatologic NLE.(ABSTRACT TRUNCATED AT 400 WORDS)
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hydrocephalus and macrocephaly new manifestations of Neonatal Lupus Erythematosus
Arthritis & Rheumatism, 2007Co-Authors: Christina Boros, Dawn Spence, Susan Blaser, Earl D SilvermanAbstract:Objective Neurologic involvement has been reported to occur in infants with Neonatal Lupus Erythematosus (NLE), although the significance of these findings is unknown. The purpose of this study was to determine if hydrocephalus/macrocephaly is a manifestation of NLE. Methods Infants from the Hospital for Sick Children in Toronto, Canada whose mothers had anti-Ro antibodies were followed prospectively. A total of 87 infants were seen in the study period (1999–2004). The maternal autoantibody status of all infants was documented, and all infants underwent full clinical examination at each visit. Results Of the 87 infants, 47 had NLE. Five of the 47 infants with NLE and 2 of the healthy infants had hydrocephalus, resulting in a prevalence of 8.0% (95% confidence interval 4.0–15.0%) in the entire cohort. This is significantly higher than the prevalence in the general population of 0.048–0.081%. Head circumference measurements of infants in the Toronto cohort were largest between 12–24 months of age (Z score difference = 0.71, P = 0.008). Conclusion We suggest that hydrocephalus and macrocephaly are manifestations of NLE and that infants born to mothers with anti-Ro antibodies should be carefully monitored for hydrocephalus as part of their routine physical examination.
Ronald M Laxer - One of the best experts on this subject based on the ideXlab platform.
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autoantibody response to the ro la particle may predict outcome in Neonatal Lupus Erythematosus
Clinical and Experimental Immunology, 2008Co-Authors: Earl D Silverman, Ronald M Laxer, Jill P Buyon, Richard Hamilton, P Bini, J L Chu, Keith B ElkonAbstract:This study was undertaken to determine the role of antibodies against both recombinant Ro (r-Ro) and La (r-La) proteins and polypeptides derived from the recombinant La protein in predicting fetal and Neonatal outcome in children at risk to develop Neonatal Lupus Erythematosus (NLE). All sera were obtained in the perinatal period and quantitative ELISA assays were used. We collected 41 maternal sera within 2 months of delivery of a child with NLE (21 with congenital heart disease block (CHB) and 20 with dermatologic NLE) and 19 sera from anti-Ro and/or anti-La antibody-positive mothers with systemic Lupus Erythematosus (SLE) who delivered a child without NLE. All sera were tested for anti-r-La and anti-r-Ro antibodies by ELISA, and most sera were tested for antibodies directed against La polypeptides by immunoblot. We found significantly higher anti-r-La antibody levels in the sera from mothers of children with NLE compared with sera from mothers of unaffected children (0.67 +/- 0.43 versus 0.14 +/- 0.30; P < 0.0001). There was a statistically significant difference in the mean anti-r-La levels between the sera of mothers of children with CHB compared with dermatologic NLE (0.51 +/- 0.45 versus 0.83 +/- 0.37 respectively; P = 0.0091). When we examined antibodies directed against the recombinant 52-kD Ro protein, there was a statistically significant elevation of titres in the sera of mothers of NLE children (0.77 +/- 0.35) compared with non-NLE mothers (0.29 +/- 0.39; P < 0.0001). There was no difference in the r-Ro levels between mothers of children with dermatologic NLE compared with CHB (0.82 +/- 0.37 versus 0.71 +/- 0.74; P = 0.32). When we examined polypeptides derived from the recombinant La protein, the mean number of polypeptides recognized by sera from mothers of children with NLE was significantly higher than the mean number of polypeptides recognized by sera from mothers of unaffected children (5.1 +/- 0.54 versus 2.3 +/- 0.54 respectively; P < 0.001). More importantly, when we examined the individual polypeptides, we found that only sera from mothers of children with NLE and not from mothers of unaffected children recognized a polypeptide designated DD (30% versus 0%, respectively). These studies indicate that the autoantibody response to the Ro/La particle can differentiate sera from mothers of children with NLE and sera from mothers of unaffected children. Furthermore, there was a difference in the anti-La autoantibody response between mothers of children with CHB and dermatologic NLE.(ABSTRACT TRUNCATED AT 400 WORDS)
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the health of mothers of children with cutaneous Neonatal Lupus Erythematosus differs from that of mothers of children with congenital heart block
The American Journal of Medicine, 2000Co-Authors: Sandra Lawrence, Ronald M Laxer, Lily Luy, Bernice R Krafchik, Earl D SilvermanAbstract:Abstract PURPOSE: Neonatal Lupus Erythematosus is caused by the transplacental passage of maternal autoantibodies. The aim of this study was to determine the risk of connective tissue disorders in mothers of children with cutaneous Neonatal Lupus Erythematosus, as compared with the risk in mothers of children with congenital heart block, which is also often caused by maternal autoantibodies. SUBJECTS AND METHODS: We prospectively studied all mothers of children with cutaneous Neonatal Lupus Erythematosus during a 14-year period at the Hospital for Sick Children, Toronto, Ontario, Canada. We identified 28 mothers, of whom 24 were eligible for study. The health and antibody status of the mothers were determined at the birth of the child and at follow-up. RESULTS: All mothers had anti-Ro antibodies at the time of birth. Initially 10 mothers were healthy and 14 mothers had either a defined (n = 9) or an undifferentiated (n = 5) autoimmune disorder. At a mean follow-up of 7 years, 13 (1 of whom had died) had a defined connective tissue disease, and 5 had an undifferentiated autoimmune disorder. Only 6 (25%) remained asymptomatic. By comparison, 36 (56%) of 64 mothers of children with congenital heart block were asymptomatic at follow-up ( P CONCLUSIONS: The majority of mothers of children with cutaneous Neonatal Lupus Erythematosus had a defined or undifferentiated autoimmune disorder at the time of the child's birth, and others developed these conditions during follow-up. The health of these mothers appears to differ from that of mothers of children with congenital heart block.
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Neonatal Lupus Erythematosus
Rheumatic Diseases Clinics of North America, 1997Co-Authors: Earl D Silverman, Ronald M LaxerAbstract:This article discusses Neonatal Lupus Erythematosus, which is a disease of the newborn defined by the presence of maternal autoantibodies and characteristic clinical features in the Neonatal period. Although the autoantibodies often are not associated with clinical disease in the mother, Neonatal Lupus Erythematosus is likely the result of fetal or Neonatal tissue damage caused by maternally transmitted IgG autoantibodies.
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importance of the immune response to the ro la particle in the development of congenital heart block and Neonatal Lupus Erythematosus
The Journal of Rheumatology, 1991Co-Authors: Edwin K Silverman, M Mamula, J A Hardin, Ronald M LaxerAbstract:Previous studies have suggested a role for anti-Ro antibodies in the pathogenesis of Neonatal Lupus Erythematosus (NLE). We reexamined the role of the immune response to the Ro/La particle in the development of NLE using newer and more definitive assay techniques. All 15 infants with congenital heart block and NLE had both anti-Ro and anti-La antibodies. Of 8 patients with cutaneous NLE alone, 2 had anti-Ro antibodies alone while the remaining 6 had both anti-Ro and anti-La antibodies. One woman gave birth to 2 children; one had both Ro and La antibodies and developed congenital heart block, the other had only anti-Ro and anti-U1RNP antibodies and was clinically normal. Our study demonstrates that the presence of anti-Ro and anti-La antibodies define the immune response that is associated with the development of congenital heart block of NLE.
Lela A Lee - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Lupus Erythematosus clinical findings and pathogenesis
The journal of investigative dermatology. Symposium proceedings the Society for Investigative Dermatology Inc. [and] European Society for Dermatologic, 2004Co-Authors: Lela A LeeAbstract:Neonatal Lupus Erythematosus is an uncommon disease associated with maternal autoantibodies to proteins of the Ro/La (SSA/SSB) family. The clinical findings most often reported are third- degree heart block and cutaneous Lupus lesions, but a significant number of children have cardiomyopathy, hepatobiliary disease, or hematologic cytopenias. The consistent presence of maternal autoantibodies and the transient nature of the disease implicate maternal autoantibodies as the cause of the disease, and developing animal models support the concept that the autoantibodies are pathogenic. Only a minority of babies exposed to the autoantibodies develop disease, however, and mothers and their babies have different disease manifestations. Thus, additional factors are likely to be important in determining disease expression.
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Neonatal Lupus Erythematosus
Journal of the Dermatology Nurses’ Association, 2002Co-Authors: Joanna M Burch, Lela A Lee, William L WestonAbstract:Neonatal Lupus Erythematosus is associated with cutaneous lesions, CHB, hepatic disease, and thrombocytopenia. IgG antibodies to Ro and/or La cross the placenta and participate in the development of the clinical manifestations. Mothers of babies with NLE are likely to develop collagen vascular diseases with time. Infants with NLE are at risk to develop other autoimmune diseases during childhood or adolescence.
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the clinical spectrum of anti ro positive cutaneous Neonatal Lupus Erythematosus
Journal of The American Academy of Dermatology, 1999Co-Authors: William L Weston, Joseph G Morelli, Lela A LeeAbstract:Abstract Background: Cutaneous Neonatal Lupus Erythematosus (NLE) is an uncommon disease described mainly through isolated case reports. Objective: Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE. Methods: This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. Results: Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital "owl-eye" or "eye mask" facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 Neonatal Lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring. Conclusion: Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.(J Am Acad Dermatol 1999;40:675-81.)
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autoantibodies of Neonatal Lupus Erythematosus
Journal of Investigative Dermatology, 1994Co-Authors: Lela A Lee, Mark Barton Frank, Victoria R Mccubbin, Morris ReichlinAbstract:Abstract The most common manifestations of Neonatal Lupus Erythematosus (NLE) are cutaneous Lupus and congenital heart block. Autoantibodies to Ro/SSA occur in almost all cases of NLE. The autoantibody response to Ro/SSA is complex, and antibodies may be detected to 60-kD Ro/SSA, 52-kD Ro/ SSA, La/SSB, and U 1 ribonuclear protein in anti-Ro/SSA-positive sera. Which of these anti-Ro/SSA-related autoantibody specificities are important in the clinical expression of NLE is not conclusively established. We examined the autoantibody specificities in 20 maternal NLE sera to determine whether autoantibody specificities correlate with the clinical findings and to evaluate the relative importance of autoantibodies to the different Ro/SSA associated proteins. Autoantibodies were examined using immunodiffusion, immunoblotting, and enzyme-linked immunosorbent assay. Eleven babies had NLE skin disease, 11 had heart block, and two had both skin disease and heart block. All 20 maternal sera had antibodies to 60-kD Ro/SSA. Eighteen of the 20 had antibodies to 52-kD Ro/SSA, nine had antibodies to La/SSB, and one had antibodies to U 1 ribonuclear protein. The prevalence of anti-La/SSB was the same in the skin-disease and heart-block subsets of NLE. Titers of anti-60-kD Ro/SSA were significantly (p These results point out the importance of 60-kD Ro/SSA as a potential target in NLE. We speculate that the lower titers of anti-60-kD Ro/SSA in the sera from mothers of babies with skin disease may be due to substantial deposition of antibodies in the mothers' and babies' skin, leading to lower circulating titers, or may reflect a lower threshold for development of skin disease than for heart block.
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Neonatal Lupus Erythematosus
Journal of Investigative Dermatology, 1993Co-Authors: Lela A LeeAbstract:Neonatal Lupus Erythematosus (NLE) is an autoimmune disease whose major findings are subacute cutaneous Lupus Erythematosus (SCLE) skin lesions and congenital heart block. Babies have maternal anti-Ro/SSA, anti-La/SSB, or anti-U1RNP autoantibodies. Anti-Ro/SSA are the predominant autoantibodies, having been found in about 95% of cases. The autoantibodies pass through the placenta from mother to child. Skin disease resolves at about the time that maternal autoantibodies can no longer be detected in the baby. NLE therefore provides the strongest clinical evidence that autoantibodies are involved in at least some manifestations of Lupus Erythematosus, but there is as yet no definitive evidence implicating autoantibodies in the disease process. Skin disease usually begins after birth, is transient, and does not result in scarring. Cardiac disease begins in utero, and the heart block is almost always permanent. Many babies require pacemakers, and about 10% die from complications related to cardiac disease. In some cases, transient liver disease or thrombocytopenia have been observed. Individuals who had NLE usually have healthy childhoods but may develop autoimmune disease in adulthood. Whether the later development of autoimmune disease is a common or an unusual event is not yet known. Mothers of babies with NLE may be asymptomatic initially, but with time usually develop symptoms of autoimmune disease. The most typical constellation of symptoms in our group of approximately 30 mothers of babies with NLE is that of Sjogren's syndrome. Most babies exposed to anti-Ro/SSA autoantibodies during gestation will not develop NLE. There is no test to determine prospectively which babies will be affected. Treatment during gestation is still controversial and, if attempted, should be reserved for fetuses with potentially life-threatening disease. Treatment after birth consists of topical management for skin disease and pacemaker implantation, if necessary, for heart block. Systemic steroids may be given for serious internal disease.
Sachiko Miyagawa - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Lupus Erythematosus a review of the racial differences and similarities in clinical serological and immunogenetic features of japanese versus caucasian patients
Journal of Dermatology, 2005Co-Authors: Sachiko MiyagawaAbstract:There has been tremendous interest in Neonatal Lupus Erythematosus (NLE) since the reports of anti-Ro/SSA antibodies as a diagnostic marker. Recent studies, including ours, have revealed racial differences as well as similarities in the clinical features and immunogenetic backgrounds of Japanese and Caucasian patients with NLE. The frequency of photosensitivity and subacute cutaneous LE lesions is not high in Japanese infants with NLE, which is in sharp contrast to their Caucasian American counterparts. The majority of Japanese infants with NLE develop annular, erythematous or edematous lesions which have also been reported in association with Sjogren's syndrome. The frequency of isolated congenital heart block (CHB) is about 50% in Japanese anti-Ro/SSA positive Neonatal Lupus infants; this is similar to the frequency among Caucasians. The HLA-DR3 phenotype, which is found in the great majority of Caucasian mothers of NLE infants, is absent in Japanese mothers. Finally, both Japanese and Caucasian children with CHB are often identical to their mothers in their alleles of HLA-DRB1, DQA1 and DQB1 loci.
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Neonatal Lupus Erythematosus results of maternal corticosteroid therapy
Obstetrics & Gynecology, 1999Co-Authors: Koji Shinohara, Sachiko Miyagawa, Tomio Fujita, Toshihiro Aono, Kinichi KidoguchiAbstract:Abstract Objective: To assess the possibility of preventing cardiac or cutaneous manifestations of Neonatal Lupus Erythematosus or treating the fetus with congenital heart block by administering corticosteroid therapy to the mother. Methods: Eighty-seven offspring of 40 anti-Ro/SSA-positive mothers, followed up from 1979 to 1996, were evaluated. Autoantibodies against Ro/SSA and La/SSB antigens were detected by immunodiffusion and enzyme-linked immunosorbent assay. Results: None of 26 neonates whose mothers received corticosteroid maintenance therapy initiated before 16 weeks’ gestation demonstrated congenital heart block, whereas 15 of 61 neonates whose mothers received no corticosteroids during pregnancy or began receiving steroid therapy after 16 weeks’ gestation had congenital heart block. Complete congenital heart block, once developed, did not respond to corticosteroid treatment in utero. Four infants whose mothers received steroid treatment before 16 weeks’ gestation had skin lesions of Neonatal Lupus Erythematosus. Conclusion: Once established, complete congenital heart block was irreversible and maternal corticosteroid therapy did not effectively prevent cutaneous Lupus Erythematosus. However, prenatal maintenance therapy with prednisolone or betamethasone given to the mother starting early in pregnancy (before 16 weeks’ gestation) might reduce the risk of developing antibody-mediated congenital heart block in the offspring.
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Neonatal Lupus Erythematosus maternal igg antibodies bind to a recombinant nh2 terminal fusion protein encoded by human α fodrin cdna
Journal of Investigative Dermatology, 1998Co-Authors: Sachiko Miyagawa, Kinichi Kidoguchi, Kumiko Yanagi, Akira Yoshioka, Toshihiko Shirai, Yoshio HayashiAbstract:IgG antibodies to a cleavage product of alpha-fodrin (120 kDa alpha-fodrin) have recently been identified as organ-specific autoantibodies in primary Sjogren's syndrome. In this study, we examined seroreactivity of mothers and infants with Neonatal Lupus Erythematosus (NLE) to a recombinant NH2-terminal protein (120 kDa alpha-fodrin) of human alpha-fodrin. Serum samples were collected during the perinatal period in seven pregnancies of five mothers delivering offspring with NLE. Anti-120 kDa alpha-fodrin antibodies were identified by immunoblotting in six of seven perinatal maternal sera of offspring with NLE: one of two congenital heart block offspring and all five offspring with cutaneous NLE. These antibodies were placentally transmitted to infants. One of the five mothers had primary Sjogren's syndrome, and four were asymptomatic. One asymptomatic mother did not demonstrate anti-120 kDa alpha-fodrin activity at the time of the first delivery of a congenital heart block infant, but was found to be positive at the time of subsequent delivery of a second child with cutaneous NLE. We propose that maternal antibodies to 120 kDa alpha-fodrin may be an additional serologic marker for the risk of NLE in anti-Ro/SS-A positive women.
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Neonatal Lupus Erythematosus hla dr and dq distributions are different among the groups of anti ro ssa positive mothers with different Neonatal outcomes
Journal of Investigative Dermatology, 1997Co-Authors: Sachiko Miyagawa, Koji Shinohara, Tomio Fujita, Kinichi Kidoguchi, Akira Yoshioka, Takaya Fukumoto, Yukio Yamashina, Kazuko Hashimoto, Shinya Sakurai, Osami NishiharaAbstract:Neonatal Lupus Erythematosus (NLE) is an antibody-mediated disorder of infants characterized by two major clinical manifestations; cutaneous Lupus lesions and congenital heart block (CHB). The disease is associated with placentally transferred maternal anti-Ro/SSA and/or La/SSB antibodies. There is a tendency for the same disease expression to occur within a sibship. To reveal a possible association of class II MHC genes with maternal anti-Ro/SSA autoimmune responses and Neonatal outcomes in NLE with a relatively homogeneous ethnic background, haplotype, and allele distributions were analyzed based on the PCR-RFLP results in 26 Japanese anti-Ro/SSA-positive mothers from three groups defined by Neonatal outcomes. The results were as follows: (i) maternal HLA-DR5 haplotype DRB1*1101-DQA1*0501-DQB1*0301 and individual class II alIeles making up this haplotype were significantly associated with Neonatal cutaneous Lupus but not CHB. Conversely, maternal HLA-DQB1*0602 carried on HLA-DR2 haplotypes was associated with CHB but not cutaneous NLE; (ii) HLA-DQA1 alleles with glutamine at position 34 of the first domain, which have reportedly been associated with the autointmune responses to Ro/SSA antigens in other ethnic groups, were increased in the mothers of infants with cutaneous involvement; and (iii) there was no particular class II HLA profile that distinguished the disease manifestations in infants. These findings suggest that specific maternal MHC class II genes might correlate with specific Neonatal outcomes in NLE.
Alan R Shalita - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Lupus Erythematosus discordant disease expression of u1rnp positive antibodies in fraternal twins is this a subset of Neonatal Lupus Erythematosus or a new distinct syndrome
Journal of The American Academy of Dermatology, 1995Co-Authors: Barry A Solomon, Teresita A Laude, Alan R ShalitaAbstract:Neonatal Lupus Erythematosus (NLE) is an uncommon disease that is manifested by cutaneous lesions, cardiac conduction defects, or both, that appear in utero or shortly after birth. In approximately 95% of patients, anti-Ro antibody (Ro[SS-A]) has been identified and has become the serologic marker for NLE. Since 1987 there have been four reported cases of Ro- and anti-La antibody (La[SS-B])-negative, U1RNP antibody-positive, NLE. Our affected twin, as well as all other infants with U1RNP-positive NLE, had cutaneous lesions similar to those in Ro-positive NLE, although they lacked systemic abnormalities, including cardiac conduction defects. HLA typing of mothers with infants with U1RNP-positive NLE revealed the presence of HLA-DR4, DQw1, or DQw3 phenotypes. Our typing confirms these findings. As with Ro-positive NLE, no distinct HLA associations were demonstrated in the infants. Unlike Ro-positive mothers, all mothers with a U1RNP-positive infant with NLE had connective tissue disease at the time of the diagnosis and had a different spectrum of disease. We describe the clinical, serologic, and immunogenetic findings in the first reported case of U1RNP-positive NLE in dizygotic twins in whom the NLE disease expression was discordant.
