RESUMO
Describe a case of a female patient having anti-Ro/SSA antibodies without any other risk factor or collagen disease. In her first pregnancy a congenital heart block and hydrops in the fetus were diagnosed, and these caused stillbirth. In a second pregnancy an in utero treatment resulted in the succesful delivery of a normal child.
Assuntos
Anticorpos Antinucleares , Doenças Fetais/imunologia , Bloqueio Cardíaco/congênito , Adulto , Feminino , Bloqueio Cardíaco/imunologia , Humanos , GravidezAssuntos
Humanos , Masculino , Feminino , Recém-Nascido , Bloqueio Cardíaco/complicações , Bloqueio Cardíaco/congênito , Bloqueio Cardíaco/epidemiologia , Bloqueio Cardíaco/imunologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Doenças Autoimunes/complicações , Doenças Autoimunes/congênitoRESUMO
OBJECTIVE: Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS: We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS: The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION: In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women.
Assuntos
Filho de Pais com Deficiência , Bloqueio Cardíaco/diagnóstico , Lúpus Eritematoso Sistêmico , Anticorpos Antinucleares/sangue , Ecocardiografia Doppler em Cores , Eletrocardiografia , Feminino , Bloqueio Cardíaco/diagnóstico por imagem , Bloqueio Cardíaco/imunologia , Frequência Cardíaca , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Estatísticas não ParamétricasRESUMO
OBJECTIVE: Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS: We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS: The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION: In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Eletrocardiografia , Bloqueio Cardíaco/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Anticorpos Antinucleares/sangue , Ecocardiografia Doppler em Cores , Frequência Cardíaca , Bloqueio Cardíaco/imunologia , Bloqueio Cardíaco , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico , Estudos Prospectivos , Estatísticas não ParamétricasRESUMO
OBJECTIVE: Neonatal lupus erythematosus (NLE) is characterized by complete congenital heart block (CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with autoantibodies directed against SSA/Ro, SSB/La, or both. We carried out a prospective study to determine the incidence of individual NLE features. STUDY DESIGN: The study was performed in two centers: Toronto, Canada, and Milano, Italy. Mothers had been referred for the presence of anti-SSA/Ro autoantibodies, regardless of their diagnosis. All the children were seen at least once within the first 6 months of life for clinical evaluation and laboratory testing. The study group consisted of 128 infants born from 124 pregnancies in 112 women with anti-Ro antibodies with or without anti-La antibodies. RESULTS: There were two cases of CCHB for an overall percentage of 1.6%. Twenty-one children (16%) developed cutaneous NLE. Laboratory testing showed hematologic abnormalities in 27% of the babies and elevation of liver enzymes in 26%. CONCLUSIONS: Mothers with autoimmune diseases and anti-Ro antibodies are at risk of delivering a child with NLE but at a low risk of delivering a child with CCHB. Infants born to mothers with anti-Ro or anti-La antibodies should be monitored for other features of NLE in addition to CCHB.
Assuntos
Autoantígenos/imunologia , Doenças Autoimunes/imunologia , Bloqueio Cardíaco/congênito , Lúpus Eritematoso Sistêmico/epidemiologia , Complicações na Gravidez/imunologia , RNA Citoplasmático Pequeno , Ribonucleoproteínas/imunologia , Adolescente , Adulto , Autoanticorpos/imunologia , Feminino , Bloqueio Cardíaco/imunologia , Humanos , Incidência , Recém-Nascido , Masculino , Gravidez , Estudos ProspectivosRESUMO
Isolated congenital heart block may be associated with autoimmune disorder such as Sjögren Syndrome and systemic lupus erythematosus. In this work we demonstrate circulating autoantibodies against neonatal heart M1 muscarinic acetylcholine receptor (mAChR) in the sera of children with congenital heart block. This antibody were able to react with the second extracellular loop of the human M1 mAChR as demonstrated using a synthetic peptide in enzyme immune assay and binding assay. Affinity purified anti-peptide IgG as well as total IgG from children with congenital heart block, interfered with the specific radioligand occupancy from neonatal heart M1 mAChR, interacting irreversibly. The antipeptide antibodies also displayed an 'agonist-like' activity, i.e. decreased contractility, activated nitric oxide synthase activity and increased production of cyclic GMP. All of these effects were selectively blunted by pirenzepine and neutralized by the synthetic M1 peptide. Both binding and biological effects were obtained using neonatal rat heart instead adult heart and were independent of Ro/SS-A and La/SS-B antibodies and were also absent in the sera of normal children. A clinical relevance of these findings is demonstrated by a strong association between the existence of circulating M1 mAChR antipeptide antibodies and the presence of isolated congenital heart block, making these antibodies a proper marker of this disease.
Assuntos
Autoanticorpos/imunologia , Autoantígenos/imunologia , Bloqueio Cardíaco/imunologia , Receptores Muscarínicos/imunologia , Sequência de Aminoácidos , Animais , Autoanticorpos/sangue , Criança , Pré-Escolar , GMP Cíclico/imunologia , Coração , Bloqueio Cardíaco/sangue , Bloqueio Cardíaco/complicações , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/imunologia , Humanos , Lactente , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/imunologia , Dados de Sequência Molecular , Óxido Nítrico Sintase/imunologia , Peptídeos/imunologia , Ratos , Receptor Muscarínico M1RESUMO
Neonatal lupus erythematosus (NLE) is an auto-immune disease related to systemic lupus erythematosus (SLE). Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflammatory reaction in the fetal tissues. NLE is the principal cause of atria-ventricular block, when it is not associated with congenital birth defects. All the clinical studies to date correlate the heart block in NLE with the presence of certain types of circulating maternal antibodies, against the Ro/SSA nuclear proteins, in the serum of the newborn. In this paper we discuss animal models that have been developed by our and others groups to study the participation of the anti-Ro/SSA antibodies in the pathogenesis of the cardiac conduction blockades that occur in NLE.
Assuntos
Bloqueio Cardíaco/congênito , Imunidade Materno-Adquirida , Lúpus Eritematoso Sistêmico/congênito , Animais , Anticorpos Antinucleares/imunologia , Autoanticorpos/imunologia , Doenças Autoimunes/imunologia , Modelos Animais de Doenças , Bloqueio Cardíaco/imunologia , Lúpus Eritematoso Sistêmico/imunologia , SíndromeRESUMO
Neonatal lupus erythematosus (NLE) is an auto-immune disease related to systemic lupus erythematosus (SLE). Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflamatory reaction in the fetal tissues. NLE is the principal cause of atria-ventricular block, when it is not associated with congenial birth defects. All the clinical studies to date correlate the heart block in NLE with the presence of certain types of circulating maternal antibodies, against the Ro/SSA nuclear proteins, in the serum of the newborn. In this paper we discuss animal models that have been developed by our and other groups to study the participation of the anti-Ro/SSA antibodies in the pathogenesis of the cardiac conduction blockades that occur in NLE.