Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Research progress on the manifestations and prognosis of neonatal lupus erythematosus in various systems / 中国当代儿科杂志

Neonatal lupus erythematosus (NLE) is caused by the transmission of maternal anti-Ro/SSA antibodies, anti-La/SSB antibodies, and other autoantibodies to the fetus through the placenta. Usually, with the disappearance of autoantibodies in the children's body, abnormal changes in the mucocutaneous, blood system, and digestive system can spontaneously subside, but the damage to various systems caused by autoantibodies may persist for a long time. This article provides a comprehensive review of the manifestations and prognosis of NLE in various systems, including mucocutaneous, blood system, circulatory system, nervous system, digestive system, respiratory system, aiming to provide reference for clinical work.

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

Lupus neonatal. Reporte de un caso

El lupus eritematoso neonatal (LEN) es una enfermedad rara, con incidencia de aproximadamente 2% en recién nacidos y menos del 5% de estos bebés desarrollarán lupus eritematoso sistémico en la adolescencia tardía o en la adultez temprana. Se presenta un caso de un recién nacido de término, hijo de madre sin antecedentes previos, sexo femenino, con marcadores inmunológicos positivos; nace con 3.280 gramos de peso, 40 semanas de edad gestacional, con lesiones en piel en el que el diagnóstico diferencial fue infección herpética, llegando al diagnóstico y logrando descartar de forma oportuna afectación sistémica, la que podría llegar a poner en riesgo la vida del neonato, con esta presentación de caso se concluye la importancia de agregar entre los diagnósticos diferenciales, el lupus eritematoso neonatal, entre los pacientes con lesión en piel incluso desde su nacimiento.

Neonatal lupus erythematosus (NLE) is a rare disease, with an incidence of approximately 2% in newborns. Less than 5% of these babies will develop systemic lupus erythematosus in late adolescence or early adulthood. A case of a female term newborn is presented, born to a mother with an unremarkable previous history, with positive immunological markers; birth weight of 3,280 grams, 40 weeks gestational age, with skin lesions in which the differential diagnosis was herpetic infection. The diagnosis was made and systemic involvement was ruled out in a timely manner, which could have potentially endangered the life of the newborn. This case demonstrates the importance of adding neonatal lupus erythematosus to the differential diagnosis in patients with skin lesions at birth.

Permanent pacemaker implantation in complete congenital fetal atrioventricular (AV) block: a case report / Implantación de marcapasos permanente en bloqueo atrioventricular (AV) fetal congénito completo: reporte de caso

Abstract: One of the most common pathologies attributed to lupus neonatal refers to atrioventricular (AV) congenital block, which diagnosis can be made between 16 and 30 weeks of gestation due to persistent fetal bradycardia. The development of this disease is mostly related to maternal anti-Ro/SSA and anti-Smith autoantibodies. Currently, there are a number of alternatives for prenatal and postnatal treatment, with some controversy about their viability. The placement of a permanent pacemaker is presented as one of the most appropriate procedures currently, even with the risks awarded. This case report describes the placement of a permanent pacemaker to a two-month-old newborn with high maternal contents of anti-Ro/SSA and anti-Smith nuclear autoantibodies, with a favorable outcome.(AU)

Resumen: Una de las patologías más comunes atribuidas al lupus neonatal se refiere al bloqueo congénito atrioventricular (AV), diagnóstico que se puede realizar entre 16 y 30 semanas de gestación debido a bradicardia fetal persistente. El desarrollo de esta enfermedad se relaciona principalmente con los anticuerpos anti-Ro/SSA materno y anti-Smith. Actualmente, existen varias alternativas para el tratamiento prenatal y postnatal, con cierta controversia sobre su viabilidad. La colocación de un marcapasos permanente se presenta como uno de los procedimientos más adecuados actualmente, incluso con los riesgos adjudicados. Este relato de caso describe la colocación de un marcapasos permanente en un recién nacido de dos meses con alto contenido materno de autoanticuerpos anti-Ro/SSA y anti-Smith, con un resultado favorable.(AU)

Two Cases of Neonatal Lupus and Literature Review

Neonatal lupus is a rare rheumatic disease. Clinical manifestations include characteristic annular or macular rashes, congenital heart block, cytopenias, and hepatitis. Neonatal lupus is caused by transmission of maternal immunoglobulin G autoantibodies such as anti-SSA/Ro antibody or anti-SSB/La antibody to the fetus through the placenta. We report two cases of neonatal lupus. The first case refers to an 18-day-old male with annular rashes on both cheeks, neutropenia, positive tests for antinuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody. His mother was diagnosed with systemic lupus erythematosus characterized by positive tests for antinuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody. The second case represents a 32-day-old female with annular rash on both hands, soles, and the genital area, neutropenia, hepatitis, positive tests for antinuclear antibody, and anti-SSA/Ro antibody. Skin punch biopsy was conducted. Her mother did not have history of connective tissue diseases. We referred her mother to the division of rheumatology of the department of internal medicine. The mother was suspected with primary Sjögren's syndrome because of arthralgia and dry eye symptoms with positive tests for antinuclear antibody, anti-SSA/Ro antibody, anti-SSB/La antibody, and rheumatoid factor. It is necessary to suspect neonatal lupus in neonates or infants with characteristic annular rash with or without maternal history of connective tissue disorders.

Telangiectasia Occurred after Neonatal Lupus Erythematosus / 대한피부과학회지

Neonatal lupus erythematosus (NLE) is an uncommon autoimmune disease that is associated with transplacental passage of maternal autoantibodies into the fetal circulation. It is commonly presented as multiple round, pink to red macules involving scalp, face and extremities. The characteristic skin lesions of NLE generally resolve without scarring within 6 months, but transient hyperpigmentation or hypopigmentation has been reported in some dark-skinned infants. We report a rare case of 3 year-old girl showing telangiectasia at the site of previous NLE lesions. In past history, at 2 days after birth, erythematous macules and patches on face and extremities had been presented and she was diagnosed as NLE in pediatrics. To our knowledge, it is the first report of residual telangiectasia after NLE in Korean dermatologic literature. Furthermore, we propose that dermatologists should consider the possibility of the cutaneous sequelae associated with NLE when encountering a child showing multiple telangiectasia.

Neonatal Lupus Erythematosus in an Infant Born to an Asymptomatic Mother with Anti-SSA/SSB Antibodies / 대한피부과학회지

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.

Neonatal Lupus Erythematosus in an Infant Born to an Asymptomatic Mother with Anti-SSA/SSB Antibodies / 대한피부과학회지

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.

Avaliação da estimulação ventricular direita crônica em crianças e adultos jovens com bloqueio atrioventricular congênito isolado / Evaluation of chronic right ventricular pacing in children and young adults with isolated congenital complete atrioventricular block

Introdução: O bloqueio atrioventricular congênito isolado (BAVCi) é raro e tem múltiplas apresentações clínicas. O implante de marca-passo cardíaco permanente (MP) é o tratamento de escolha, resultando em evolução clínica satisfatória para a maioria dos casos, porém, aproximadamente 10% deles apresentam remodelamento ventricular e insuficiência cardíaca grave. Objetivos: Estudar a evolução tardia de crianças e adultos jovens com BAVCi e estimulação crônica do ventrículo direito (VD), visando determinar: a prevalência de sinais clínicos e laboratoriais de insuficiência cardíaca e de remodelamento ventricular; a capacidade funcional; a qualidade de vida e fatores preditores de alterações clínicas, funcionais ou ecocardiográficas. Métodos: Estudo transversal realizado em coorte de portadores de BAVCi e MP implantado antes de 21 anos de idade com estimulação no VD há mais de um ano. Todos os indivíduos foram submetidos a avaliação clínica e laboratorial, da capacidade funcional, da qualidade de vida e a ecocardiograma. Mães e sujeitos da pesquisa foram investigados para doenças reumatológicas. Os dados foram armazenados no sistema REDCap (Research Electronic Data Capture) e analisados pelos programas SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) e R Studio. A análise dos dados incluiu: análise univariada para pesquisa de associações entre variáveis preditoras e desfechos, coeficiente de correlação de Pearson e modelo de regressão linear multivariado. Resultados: De março/2010 a dezembro/2013, foram avaliados 63 indivíduos, 68% do sexo feminino, com idade de 1 a 40 anos, com MP por 13,4 ± 6,5 anos e estimulação do VD por 10,0 ± 5,4 anos. O modo de estimulação era atrioventricular em 55,6%, o percentual de estimulação de VD de 97,9 ± 4,2% e a duração do complexo QRS estimulado de 152,4 ± 20,1 ms. A maioria (88,9%) era assintomática e não utilizava medicamentos de ação cardiovascular. Maior tempo de MP...

Introduction: Isolated congenital atrioventricular block (iCAVB) is a rare condition with multiple clinical presentations. Permanent cardiac pacing is the most effective therapy for this population resulting in satisfactory long-term outcomes. However, approximately 10% of patients may have ventricular remodeling and severe heart failure. Objectives: To study the long-term effects of chronic right ventricular (RV) pacing in children and young adults with iCAVB in order to determine: prevalence of clinical and laboratory signs of heart failure and ventricular remodeling, functional capacity, quality of life and predictors of clinical, functional or echocardiographic abnormalities. Methods: Cross-sectional study of a cohort of iCAVB patients with <= 21 years old at initial pacemaker (PM) implantation and single or dual-chamber pacing in a unique RV site for a minimum of one year. All subjects underwent clinical and laboratory assessment, functional capacity, quality of life and echocardiogram. Mothers and research subjects were investigated for rheumatic diseases. Data were stored in REDCap (Research Electronic Data Capture) system and analyzed by SAS (Statistical Analysis System), SPSS (Statistical Package for the Social Sciences) and R Studio programs. Data analysis included: univariate analysis for associations between predictor variables and outcomes, Pearson correlation coefficient and linear regression multivariate model. Results: Between March/2010 and December/2013, we evaluated 63 subjects aged 1-40 years old, 68% female, under PM for 13.4 ± 6.5 years and under RV pacing for 10.0 ± 5.4 years. Pacing mode was atrioventricular in 55.6%, percentage of RV pacing was 97.9 ± 4.2% and paced QRS duration was 152.4 ± 20.1 ms. Overall, the majority (88.9%) were asymptomatic and did not use cardiovascular drugs. Longer time under PM (P= 0.013), or even under RV pacing (P= 0.005), higher age at study inclusion (P= 0.032) and lower left ventricular...

An Unusual Case of Severe Neonatal Lupus Mimicking Neonatal Sepsis and Literature Review

Neonatal lupus is an uncommon autoimmune disease that results from transplacental passage of the maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Pancytopenia or severe jaundice is a rare manifestation of neonatal lupus, respectively, and could be misdiagnosed with other neonatal illnesses, such as infection, hematologic disease, or hepatobiliary disease. Here, we report an unusual case of a premature newborn with severe neonatal lupus manifested with skin rash, fever, pancytopenia, and severe jaundice with abnormal liver function tests. His mother had been clinically asymptomatic before delivery; however, she revealed peripheral edema, bilateral pleural effusion, and ascites after delivery and diagnosed with systemic lupus erythematosus based on positive anti-nuclear and anti-cardiolipin antibodies, proteinuria, and serositis. The newborn and his mother had anti-SSA/Ro and anti-SSB/La antibodies. His pancytopenia and jaundice were progressively aggravated, and his illness was confused with neonatal sepsis or biliary obstruction. We decided to treat with high dose of corticosteroid and intravenous immunoglobulin, and he gradually recovered completely with the treatment. His corticosteroid was stopped at 5 months of age without relapse or complication.

Neonatal Congenital Heart Block.

Congenital Heart Block (CHB) is the most serious complication of neonatal lupus erythematosus. Transplasental transfer of maternal anti-SSA/Ro or anti-SSB/La antibodies around 12th week of gestation is associated with development of CHB. This may lead to inflammation, fibrosis and scarring of fetal conduction system in the early second trimester. Different degrees of atrioventricular (AV) block may be seen in the affected fetus. First and second-degree AV blocks may change in severity; however, third degree AV block is irreversible. CHB is mostly diagnosed between 18- 24th weeks of gestation. Even if most of the mothers carrying autoantibodies of several rheumatic diseases such as systemic lupus erythematosus or Sjogren’s syndrome are not aware of their diseases until their children are born with CHB, it is recommended that antibody-positive mothers or the mothers having babies with neonatal lupus erythematosus should be referred for close fetal echocardiographic surveillance beginning from the early second trimester. Although their utility is still controversial, various therapeutic regimes such as sympathomimetic, plasmapheresis, steroids, intravenous immunoglobulin, digoxin, diuretic and in utero pacing have been used for intrauterine treatment of CHB. Aggressive medical treatment is coupled with pacing in infants who do not respond to medical therapy alone.

Research progress on neonatal lupus erythematosus / 临床儿科杂志

This review summarizes the epidemiology, etiology, clinical manifestation, treatment, follow-up of neonatal lupus erythematosus with focus on new discoveries on the etiology of the disease in recent years including anti-SSA/Ro and anti-SSB/La antibodies, serotonin (5-hydroxytryptamine 4), apoptosis of cardiac cells, calcium channels, maternal micro-chimera, genetic variants, to improve clinician awareness of the disease.

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.

A Case of Pacemaker Implantation in Premature Newborn with Congenital Complete Atrioventricular Block

The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.

Neonatal Lupus Erythematosus Presenting with "Eye Mask" Facial Rash and Telangiectasia / 대한피부과학회지

Neonatal lupus erythematosus (NLE) is an autoimmune disease associated with the maternal anti-SSA/Ro and anti-SSB/La antibodies. NLE is characterized by cutaneous erythema, congenital heart block (CHB), hepatic dysfunction and hematological abnormalities. The characteristic cutaneous lesion is an annular or polycyclic erythematous patch or plaque. Individual annular lesions are often about 1 cm in diameter. We report a case of NLE in a 7-day-old female infant, born of a clinically asymptomatic mother, presenting "eye mask" shape rash and telangiectasia on her face. Periorbital rash with "eye mask" appearance presents in a few of NLE cases, and this cutaneous sign has been emphasized as a diagnostic clue to NLE. Her serological tests revealed high titers of anti-SSA/Ro, anti-SSB/La and antinuclear antibody. Based on these clinical and serological findings, the patient was diagnosed as NLE with characteristic periorbital skin findings.

A Case of Neonatal Lupus Erythematosus and Retrospectively Diagnosed with Asymtomatic Maternal Lupus Erythematosus

Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.

Cutaneous Neonatal Lupus Erythematosus Associated with Brain Infarction

Neonatal lupus erythematosus (NLE) is an acquired autoimmune disorder caused by the transplacental passage of maternal autoantibodies, usually anti-Ro/SSA or anti-La/SSB antibodies, and less commonly U1-ribonucleoprotein. NLE usually involves a single organ, but multiple organ involvement has also been reported. Manifestations of NLE may include cutaneous lesions, congenital heart block, hematological diseases (anemia, thrombocytopenia, neutropenia), hepatic diseases (hepatitis, hepatosplenomegaly, cholestasis), and neurological diseases. Neurological involvement is very rare in infants with NLE. Here, we report a 2-day-old female neonate, born to a clinically asymptomatic mother, presenting with cutaneous lupus lesions and brain infarction as a neurological disease.

Lúpus eritematoso sistêmico e gestação: série de casos com diferentes evoluções / Systemic lupus erythematosus and pregnancy: cases series with different evolution

JUSTIFICATIVA E OBJETIVOS: O lúpus eritematoso sistêmico (LES) é uma doença inflamatória crônica, caracterizada pela produção de autoanticorpos voltados contra antígenos nucleares e outros antígenos celulares, consistindo desta forma em desequilíbrio imunológico que afeta principalmente pacientes jovens do sexo feminino. A associação de lúpus e gravidez é frequente, o que contribui para o aparecimento de complicações durante a gestação. O objetivo deste estudo foi relatar quatro casos de LES associados à gestação, buscando esclarecer os aspectos da doença e correlacionar os dados obtidos na literatura com o quadro apresentado pelas pacientes.RELATO DOS CASOS: São descritas quatro evoluções diferentes, sendo apresentado um caso de síndrome do anticorpo antifosfolípide, outro caso de síndrome do lúpus neonatal, outro de prematuridade e caso com evolução para óbito materno e fetal.CONCLUSÃO: Após correlacionar a literatura e os casos constataram-se a importância do acompanhamento e tratamento adequado das pacientes gestantes portadoras de LES, na tentativa de redução da morbimortalidade materna e fetal.(AU)

BACKGROUND AND OBJECTIVES: Systemic lupus erythematosus (SLE) is a chronic inflammatory disease characterized by production of auto antibodies against the nuclear antigens and other cellular antigens, with immune imbalance in this way that affects mainly young female patients. The association of lupus and pregnancy is common, which contributes to the appearance of complications during pregnancy. The purpose of this study is to report four cases of SLE associated with pregnancy, seeking to clarify aspects of the disease and correlate the data obtained in the research literature, to the table for patients.CASE REPORTS: We describe four different developments, and presented a case of antiphospholipid antibody syndrome, one case of neonatal lupus syndrome, one case of prematurity and progression to death with maternal and fetal.CONCLUSION: After correlating the literature and the cases it was observed the importance of monitoring and appropriate treatment of pregnant patients suffering from SLE, in an attempt to reduce morbidity and maternal and fetal mortality.(AU)