Congenital heart block secondary to neonatal lupus

@#Neonatal lupus is a passively acquired autoimmune disease that occurs in children of mothers with anti-Ro/SSA and/or anti-La/SSB antibodies. [1-4] The most serious complication in the neonate is complete heart block. [3-8] This is a case report of a newborn female presenting with persistent bradycardia detected in utero. The diagnosis was confirmed by maternal anti-Ro/SSA and/or anti-La/SSB antibodies and in utero detection of fetal heart block on echocardiogram. Therapeutic management involved placement of a permanent pacemaker.

Lupus eritematoso neonatal. Reporte de caso de lactante concebida por inseminación artificial y madre con artritis reumatoidea / Lupus eritematoso neonatal Report of the infant case conceived by artificial insemination and mother with rheumatoid arthritis

El Lupus Eritematoso Neonatal es una enfermedad de origen autoinmune caracterizada por rash cutáneo transitorio, bloqueo cardíaco congénito permanente, función hepática anormal con o sin enfermedad biliar y compromiso hematológico asociado a la presencia de autoanticuerpos maternos contra la ribonucleoproteinas solubles (SSB/La, SSA/Ro y Anti-RNP). Se presenta el caso de una niña de cinco meses de edad con hallazgos clínicos e histopatológicos de Lupus eritematoso neonatal. Es una condición que no suele dejar secuelas, aunque se han reportado casos de atrofia cutánea e hiperpigmentación

Neonatal Lupus Erythematosus is an autoimmune disease characterized by transitory cutaneous rash, congenital heart block, abnormal liver function test with or without cholestasis and hematologic features associated to anti-Ro and anti-La autoantibodies. A 5-month-old female is brought to the hospital with clinical and histopathology findings of Neonatal lupus. This medical condition does not leave long term physical damage, however there have been cases reported with cutaneous atrophy and hyperpigmentation

Miocarditis y bloqueo aurículo-ventricular fetal asociado a lupus eritematoso sistémico materno / Myocarditis and atrioventricular block associated with systemic lupus erythematosus

El lupus eritematoso sistémico (LES) es una de las patologías autoinmunes más frecuentes durante el embarazo, asociándose con distintas complicaciones fetales y neonatales, sobre todo cardíacas, secundario al traspaso de anticuerpos maternos a través de la placenta. Estos anticuerpos se unen a los cardiomioci-tos fetales, desencadenando una respuesta inflamatoria local que determina la aparición de lesiones que pueden ser permanentes y letales. Presentamos el caso de una paciente embarazada con LES, en la cual se observó en el feto la presencia de bloqueo aurículo-ventricular de primer grado y signos sugerentes de miocarditis. Estas complicaciones se caracterizan por un aumento en la morbimortalidad perinatal, por lo que las estrategias actuales están dirigidas a la detección precoz de éstas y también en la prevención de las mismas. Un tratamiento estándar aun es tema de investigación, pese a los reportes que muestran la efectividad de corticoides como la dexametasona. En embarazadas con anticuerpos anti-Ro positivo se recomienda efectuar ecocardiograma fetal seriados cada 1-2 semanas desde la semana 16, para detectar precozmente anomalías cardiacas sobre las cuales pudiese intervenirse.

Systemic lupus erythematosus (SLE) is one of the most common autoimmune disease during pregnancy, associated with various fetal and neonatal complications, especially heart disease, secondary to the transfer of maternal antibodies through the placenta. These antibodies bind to fetal cardiomyocytes, triggering a local inflammatory response that determines the appearance of lesions that may become permanent and deadly. We report a pregnant patient with SLE, in which was observed the presence of atrioventricular block of 1st degree and signs suggestive of myocarditis in the fetus. These complications are characterized by an increase in fetal and neonatal morbidity and mortality, so that current strategies are aimed at early detection of these and also in preventing them. A standard therapy for atrioventricular block is still matter of investigation, although corticosteroids like dexamethasone have been reported to be effective for associated cardiomyo-pathy. Serial echocardiograms and obstetric sonograms, performed at least every 1-2 weeks starting from the 16th week of gestational age, are recommended in anti-Ro/SSA-positive pregnant women to detect early fetal abnormalities that might be a target of preventive therapy.

Bloqueo cardiaco fetal completo: reporte de dos casos y revisión de la literatura / Fetal complete atrioventricular block: two case report and literature review

Presentamos 2 casos de embarazos controlados en nuestro servicio con el diagnóstico de bloqueo aurículo-ventricular fetal. Este es un tipo de arritmia poco frecuente, relacionado con la presencia de anticuerpos antiribonucleoproteínas (Ro y La). El manejo es expectante en la mayoría de los casos ya que no existe forma de revertir el bloqueo; en caso de evidenciar una descompensación hemodinámica fetal, se pueden administrar corticoides como medida terapéutica con un éxito limitado. No existe contraindicación del parto vaginal y el uso de pH de cuero cabelludo y oximetría de pulso parecen ser métodos adecuados para la evaluación de la condición fetal intraparto. Recomendamos el enfoque multidisciplinario en esta patología para evitar intervenciones innecesarias, anticipar los riesgos fetales y obtener un mejor pronóstico postnatal.

Here we report the perinatal outcome of two patients with fetal complete atrioventricular (AV) block. This is an uncommon disease, related to the presence of autoantibodies against ribonucleoproteins (Ro and La). Management should be expectant in most cases because a treatment to revert the AV block is not available; when fetal hemodynamic problems are detected corticosteroids can be used, but with limited effectiveness. Vaginal delivery is allowed; fetal scalp pH and pulse oximetry are appropriate for intrapartum fetal surveillance. We recommend a multi disciplinary approach to avoid unnecessary interventions, anticipate fetal risk and obtain a better perinatal outcome.

Anti-La negative cutaneous lupus erythematosus in a newborn.

A newborn presented with erythematous lesion over face, which appeared soon after birth. Diagnosis of neonatal lupus erythematosus (NLE) was confirmed by positive anti-Ro SSA antibody and skin biopsy. But anti-La SSB antibody was negative. Her hepatic transaminases were high. But no cardiac manifestations were noted.

Neuromyelitis Optica With Positive Anti-Ro and Anti-La Antibodies

No abstract available.

A Case of Cogan's Syndrome with Antibodies to Ro (SS-A) and La (SS-B) Antigen / 대한류마티스학회지

Cogan's syndrome is a rare chronic inflammatory disease of unknown origin, characterized by nonsyphilitic interstitial keratitis, vestibuloauditory dysfunction and vasculitis. Cogan's syndrome is uncommon and few cases have been published. A case of Cogan's syndrome in a patient with anti-Ro and anti-La positivity is described. A 24-year-old woman visited to department of rheumatology with dry mouth, dry eye, and vertigo on August 2004, and interstitial keratitis had developed on October 2004. She was admitted to the otolaryngology department with rapidly progressive hearing loss on December 2004. The patient's audiogram revealed severe sensorineural deafness. The patient was treated with systemic corticosteroid and methotrexate. This case is the first to report a case of Cogan's syndrome associated with anti-Ro and anti-La positivity.

A Case of Neonatal Lupus Erythematosus / 대한피부과학회지

Neonatal lupus erythematosus is a rare disorder caused by the transplacental passage of maternal autoantibodies, which result in congenital heart block or cutaneous eruptions similar to lesions of subacute cutaneous lupus erythematosus. We report a case of a 29 day-old-male infant who presented with multiple, targetoid lesions with an edematous margin on the scalp, face, neck, and extremities. His serological tests revealed high titers of anti-La/SSB and antinuclear antibodies. His healthy mother also showed the same high titer of antibodies. Yet, the anti-Ro/SSA antibodies were negative in both of them.

A Case of Neonatal Lupus Syndrome with Acute Myocarditis

Neonatal lupus is a model of passively acquired autoimmunity in which immune abnormalities in the mother lead to production of antibodies that cross the placenta and injure the developing fetus. The serologic markers for the diagnosis of neonatal lupus are auto-antibodies specific to SS-A/Ro and/or SS-B/La. More than 95% of affected infants are anti-SS-A/Ro positive. We experienced a neonatal lupus with acute myocarditis at 2 months of age. The baby was born in gestational age of 36 weeks with a birth weight of 2,350gm by Caesarean section. At birth, chest X-ray showed mild cardiomegaly, but electrocardiography and echocardiography were normal. Laboratory findings for LDH, CPK and CK-MB were increased above the normal range. At 2 months of age, controlled echocardiography showed dilated left ventricle(LV) dimension with decreased fractional shortening and ejection fraction. However, this infant did not show clinical symptoms of congestive heart failure. We followed up on this patient without giving any medical treatment. At 1 year of age, controlled echocardiography showed increased thickness of the posterior wall of LV, but the fractional shortening and ejection fraction returned to normal range.

A Case of Neonatal Lupus Syndrome with Congenital Heart Block

Neonatal lupus erythematous is a rare syndrome, which is characterized by a transient lupus dermatitis, hematologic abnormalities and isolated congenital heart block. We detected the atrioventricular dissociation, bradycardia and pericardial effusion by fetal echocardiography in a female fetus at 25th weeks of gestational age. The baby was born in the 38th week of pregnancy by Cesarean section with 1790 gram of body weight. In spite of atrioventricular dissociation with bradycardia and pericardial effusion, cardiac pacemaker was not needed during neonatal period because she was doing well, no evidence of congestive heart failure and around 80 beat per minute of heart rate. The serologic markers for diagnosis of neonatal lupus are the autoantibodies specific to SS-A/Ro and/or SS-B/La. These antibodies are produced by the mother and passed to the fetus through the placenta. Autoantibodies of cytoplasmic SS-A antigens or SS-B antigen were found in the blood of this patient and her mother. We report a case of neonatal lupus syndrome with congenital atrioventricular dissociation with Rt. Bundle branch block and hematologic abnormality.