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IntroductionThere are few reports of severe hematological involvement in children with neonatal lupus erythematosus (NLE) treated with exchange transfusion. In this case report, we present a female patient with NLE admitted to the Children's Hospital of Soochow University. The main clinical manifestations were pancytopenia and congenital heart block (CHB). Her condition was serious and could not be improved by conventional treatment; however, she responded well to exchange transfusion therapy.Case presentationA female infant, aged 1 month and 3 days, was admitted to the Children's Hospital of Soochow University owing to the “discovery of thrombocytopenia over 1 month.” She tested positive for anti-SSA IgG, anti-Ro-52 IgG, and anti-mitochondrial M2 antibodies. In contrast, her mother tested positive for ANA (1:320) and anti-Ro/SSA antibodies. The patient was diagnosed with NLE and presented with pancytopenia and CHB. Her cardiac function was normal and no intervention was performed; however, her hematological involvement was more severe, without significant improvement after steroid, intravenous immunoglobulin, and transfusion treatments. After exchange transfusion therapy, the patient significantly improved, and the short-term follow-up prognosis was good.ConclusionFor patients with NLE presenting with hematological involvement that cannot be improved by conventional treatment or whose condition is serious, exchange transfusion therapy should be considered to reduce antibody titers and improve their condition.

Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.

Abstract Background It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. Case presentation A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring. Physical examination revealed a bluish-purple discoloration around the lips and an irregular heartbeat. EGG showed the presence of isolated P (142 bpm) and QRS (78 bpm) waves, ventricular escape beats, and a diagnosis of NL-CHB. To reverse the condition, transcutaneous pacing and five sessions of plasmapheresis were performed. At a 1.5-year follow-up, the baby exhibited well-developed cardiac structure and normal neurodevelopment. Conclusions Transcutaneous pacing and repeated plasmapheresis might be possible to reverse CHB in NL.

This article presents the case of a 27-year-old female patient with idiopathic congenital complete heart block who does not consent to the implantation of a cardiac pacemaker but was referred by her primary care physician for cardiological evaluation. The conduction disturbance was recognized at the age of 6 and was asymptomatic. The professional disqualification from pacemaker implantation included a detailed history of a patient’s symptoms, an echocardiographic assessment of the heart, exercise testing and ECG Holter monitoring. The aid of salbutamol administered orally was also useful.

This study, conducted by searching keywords such as “maternal lupus”, “neonatal lupus”, and “congenital heart block” in databases including PubMed and Scopus, provides a detailed narrative review on fetal and neonatal lupus. Autoantibodies like anti-Ro/SSA and anti-La/SSB may cross the placenta and cause complications in neonates, such as congenital heart block (CHB). Management options involve hydroxychloroquine, which is able to counteract some of the adverse events, although the drug needs to be used carefully because of its impact on the QTc interval. Advanced pacing strategies for neonates with CHB, especially in severe forms like hydrops, are also assessed. This review emphasizes the need for interdisciplinary care by rheumatologists, obstetricians, and pediatricians in order to achieve the best maternal and neonatal health in lupus pregnancies. This multidisciplinary approach seeks to improve the outcomes and management of the disease, decreasing the burden on mothers and their infants.

About 2% of mothers with Sjögren’s syndrome and about 1% of mothers with systemic lupus erythematosus deliver a baby with a congenital heart block (CHB). This is thought to be as a result of the maternal autoantibodies that cross the placenta and cause congenital lupus in the fetus/neonate. Among patients with a 2nd or 3rd degree atrioventricular block, the mortality rate in the neonatal period is about 10%, and most neonates who survive require a pacemaker into adulthood. Despite the compelling mortality and morbidity, the data on the optimal preventive treatments are meager and not well-established. In addition to pharmaceutical therapy, one potentially effective therapy is plasmapheresis. Plasmapheresis is safe in pregnancy, well tolerated, and is effective in removing the offending substances in the serum which may cause disease. We review this literature, in order to educate the reader and to motivate interest in studying this condition in the future.

Cardiac electrical stimulation in children usually is needed in the setting of complete congenital atrioventricular block, atrioventricular block after heart surgery, and bradycardia associated with some specific channelopathies. In cases of atrioventricular block, the high percentage of ventricular stimulation raises concern on the deleterious effects of chronic stimulation of the right ventricle. In recent years, physiologic stimulation has developed as a valid approach for adult patients and a great interest has risen in offering conduction system pacing also to the pediatric population. We present three pediatric cases of stimulation of the conduction system (His bundle or left bundle branch), in order to show the intrinsic particularities and challenges implied in these new techniques.

Objective Maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies are a risk factor for congenital complete heart block (CHB). Because detailed analysis of the incidence of CHB after 24 weeks of gestational age (GA) is lacking, we aimed to ascertain the risk of “later-onset” CHB among offspring of SSA/SSB-positive mothers in the published literature. Study Design Using search terms “neonatal lupus heart block” and “autoimmune congenital heart block” on PubMed and Ovid, we gathered prospective studies of SSA/SSB-positive mothers with fetal echo surveillance starting from before CHB diagnosis and retrospective cases of fetal CHB diagnosis after 24 weeks of GA (if there was prior normal heart rate) or after birth. Results Ten prospective studies included 1,248 SSA/SSB-positive pregnancies with 24 cases of CHB diagnosed during pregnancy (1.9%). Among these, three (12.5%) were after 24 weeks—at weeks 25, 26, and 28. Our retrospective studies revealed 50 patients with CHB diagnosis in late fetal life and neonatal period and 34 in the nonneonatal childhood period. An additional four cases were diagnosed after age 18 years. Conclusion Later-onset autoimmune CHB in offspring of SSA/SSB-positive mothers does occur. Our analysis suggests that prenatal surveillance should continue beyond 24 weeks of GA but is limited by inconsistent published surveillance data.

Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease caused by transplacental passage of maternal circulating anti-Ro/SSA and anti-La/SSB antibodies which can bind to fetal cardiac tissue, damaging conduction tissues by inflammation and fibrosis. Approximately 2% of pregnancies with positive anti-Ro antibodies will be complicated by fetal atrioventricular block and the risk of recurrence in subsequent pregnancies is 10 times higher. We report a case of a clinically asymptomatic patient diagnosed with anti-Ro antibodies who had two pregnancies complicated by CHB with different outcomes. Despite preventive treatment with hydroxychloroquine (HCQ) from 6 weeks of pregnancy onward, the fetus developed second to third degree CHB. Dexamethasone was added. The pregnancy evolved to near-term with persistent intermittent CHB. It is not clear how pregnancies with recurrent fetal CHB despite prophylaxis with HCQ should be managed and there is a need for controlled studies to answer the remaining questions in relation to this subject.

Anti-Ro antibodies are detected frequently in the general population, but more so in patients with autoimmune conditions as Sjögren’s syndrome and systemic lupus erythematous (SLE). During pregnancy, anti-Ro antibodies can cross the placenta by hijacking physiological mechanisms and can have deleterious effects on the fetus. Administration of hydroxychloroquine (HCQ) to pregnant women with documented anti-Ro antibodies has been shown to prevent congenital heart block (CHB ). Serial fetal ultrasound scans and echocardiograms are controversial in pregnant women with anti-Ro antibodies. When complete CHB is diagnosed, this is irreversible and can lead to fetal heart failure, hydrops, and death. After delivery, babies with complete CHB require pacemaker. In the presence of maternal anti-Ro antibodies, there is a high risk of recurrence of CHB for future pregnancies, if there is a previously affected child. Adequate counselling and prophylactic treatment with HCQ should be encouraged.

Background: Congenital heart defects are the most common of birth defect, which leads to neonatal death after birth. Early diagnosis during prenatal period would be a benefit for precaution and treatment. Antegrade Late Diastolic Arterial blood Flow (ALDAF) was reported to measure fetal atrioventricular (AV) time intervals (FAVTI) at an early gestational ages (GA) of 6 weeks. There has been no previous studies reporting reference value of fetal atrioventricular time intervals (FAVTI) derive from ALDAF technique. Methods: Using fetal echocardiogram, this cross-sectional study was performed on 528 healthy fetuses between 14 and 40 weeks. Pulsed wave Doppler-derived FAVTI (milliseconds) were measured from ALDAF-AO and ALDAF-PA and left ventricle (LV) In/Out. Correlations between these three Doppler measurement techniques were examined with the Bland-Altman analysis and Pearson correlation coefficient. GA was used as specific reference value and its correlation with FAVTI was examined with linear regression. Results: We establish reference values of fetal atrioventricular (AV) time intervals (FAVTI) from antegrade late diastolic arterial blood flow (ALDAF) aorta (AO) and pulmonary artery (PA) from 14 to 40 weeks of gestation (GA). A positive correlation between FAVTI and GA was identified when using each of the three measurements (ALDAF-AO/ALDAF-PA and LV In/Out) (R2 = 0.177–0.272; P < 0.001). GA had the strongest impact on ALDAF-AO FAVTI, which was estimated to have a predicted FAVTI of 1.02 × GA (weeks) + 87.82. Bland-Altman analysis showed FAVTI of ALDAF-AO and ALDAF-PA were also significantly correlated (R2 = 0.573, P < 0.001). Intra-observer and inter-observer reliability coefficients showed good reproducibility (ICC >0.90) for all methods. Conclusions: This is the first study to establish reference ranges for FAVTI obtained from ALDAF-AO/ALDAF-PA for each week of gestation from 14 to 40 weeks. Our findings inform clinical practice by establishing GA-specific ALDAF-AO/PA cut-off values for the diagnosis of congenital heart block. FAVTI from ALDAF-AO/ALDAF-PA is a more practical measurement to use in the clinical setting because it is easier to investigate than LV In/Out. Good reproducibility in FAVTI measurements and a lack of fetal heart rate influence underpin the strength of our findings.

Congenital heart block (CHB) is a cardiac disease with an overall prevalence of approximately1:20000 live births. Structural heart defects, congenital malformations of the cardiac conduction system, maternal connective tissue disorders and fetal myocarditis are the main entities that can cause CHBs. Here we report three cases of congenital heart blocks due to three different pathologies and their outcomes. In the first case, we could not detect a structural pathology for congenital heart block; however, in second and third cases there were significant structural cardiac defects that could contribute to the fetal bradycardia. Prenatal management of fetal arrhythmia may improve the outcome of an affected fetus or neonate. Precise and timely prenatal diagnosis is critical for the selection of the appropriate prenatal treatments. However, there are many limitations in managing CHB even today and more useful strategies need to be investigated.

Epicardial pacing wire (EPW) insertion is an integral part of open heart surgery. However, the use of EPW insertion is also associated with complications such as bleeding, tamponade, arrhythmias, and occasionally even death of the patient. Various techniques have been described for preparing, placing, and removing EPW. We present our simple, yet effective technique of preparing, inserting, and removing EPW where the incidence of complications is nil.

Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes.

Sjögren’s syndrome (SjS) is a systemic autoimmune disease, most common in middle-aged women, characterized by chronic inflammation of exocrine glands. It can present by itself or alongside other autoimmune diseases. Main symptoms are dry eyes and mouth, but the disease can affect joints, lungs, kidneys, peripheral nervous system (PNS), and rarely central nervous system (CNS). SS-A antibodies in SjS can pass the fetoplacental barrier and cause congenital heart block. Patients with SjS are at higher risk of developing lymphoma.

A female newborn, born at 38 weeks from an uncomplicated pregnancy in a healthy 36-years-old mother, presented at birth with desquamative erythematous plaques with irregular borders, distributed bilaterally in the orbital, nasal and malar regions. Although there was no history of maternal autoimmune disease, neonatal lupus (NL) was suspected. Maternal and newborn screenings were positive for Sjögren syndrome type A antigen (anti-Ro/SSA) antibodies. No other alterations of NL were found in the newborn. Rheumatologic consultation on the mother showed no other alterations besides the antibodies. The newborn was discharged on day 3 of life without treatment and recommendations to avoid sun exposure. Outpatient follow-up was ensured in neonatology, dermatology and pediatric cardiology. The rash resolved during the first year of life, leaving slight local skin atrophy. NL is a rare transferred autoimmune disease with an incidence estimated as 1:20,000 live births. It occurs due to placental transfer of maternal autoantibodies. The major manifestations are cardiac and cutaneous, but hepatic, hematologic or neurologic findings may also be present. The rash usually affects the face and scalp and may be present at delivery but more often develops later, after exposure to ultraviolet light. It usually resolves within the first year of life without sequelae. NL is the leading cause of congenital heart block, but if it is not present at birth it rarely develops. Some mothers do not have a known autoimmune disease at the time of birth but may develop it later in life. Despite NL being a passively acquired autoimmune disease, the child is at increased risk of rheumatologic disease in childhood or adolescence.

Abstract Background To report first clinical experience on three cases of congenital complete heart block and the use of a pacemaker system with a maximum lower rate interval of 95 beats per minute. Methods We retrospectively analyzed three patients treated with a pacemaker system with a maximum lower rate interval of 95 beats per minute suffering from congenital complete heart block. We report a follow up period of 2.9 years, focusing on the patients’ growth, development, and adverse events, as well as pacemaker function. Results In all three patients pacemaker function was impeccable, including minute ventilation sensor rate adaption. All patients showed limited growths as expected, adequate development, good feeding tolerability and circadiane heart rate adaption. One patient experienced skin traction and revision. All patients showed high aortic velocity time integral values after birth. Conclusion The use of a pacemaker system with a maximum lower rate interval of 95 beats per minute in infants suffering from congenital complete heart block and showing high aortic VTI values seems to be feasible and to result in limited growths but adequate development.

We report a term female infant with congenital heart block and total anomalous of pulmonary venous return. The results of single nucleotide polymorphism oligonucleotide microarray analysis showed an interstitial duplication of approximately 818 Kb, which involved 11 genes, including the entire LAMB3 gene which is known to associate with cardiac conduction defect. Our report adds to the collective knowledge that the cardiac conduction defect is a clinical feature of chromosome 1q32.2 duplication.

Objective: To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases.Methods: This was a retrospective observational study of 2 fetuses and 14 children who presented with autoantibody-mediated arrhythmia and/or cardiomyopathy in our hospital from September 2010 to December 2018.Results: In total, 16 patients were identified, including 2 fetuses, and 14 children. One mother suffered from Sjogren's syndrome, two suffered from systemic lupus erythematosus (SLE), and the remaining 13 were asymptomatic carriers of autoantibodies. Two fetuses were diagnosed with complete congenital heart block (CHB) and had mean heart rates of 45 and 50 bpm. In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. None of the children received pacemaker implantation. During the follow-up, one 3-month-old girl who had complete CHB, DCM, and Torsades de pointes almost recovered after the administration of prednisone for ~8 years. Three cases with complete CHB had no improvement after 3–5 years of follow-up. One case with EFE and three cases with CLBBB and DCM were in stable condition now. Children with QTc prolongation and junctional ectopic tachycardia returned to a regular rhythm.Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are severe complications related to maternal autoantibodies, and the administration of steroid may be beneficial in reversing complete CHB.

Objective: Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge.Method: Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) in utero or neonatal period (up to 27 days after birth).Result: Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected in utero (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 in utero, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (p = 0.005, OR > 100; CI 95% 2.88->100 and hydrops (p = 0.003, OR = 14.09; CI 95% 2.01–122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins.Conclusion: This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.

Sjogren's syndrome is one of the most common autoimmune disorders and has a female predominance. Maternal circulating autoantibodies such as anti-Ro/SSA and anti-La/SSB antibodies can cause congenital heart block of fetus, and in severe case, emergency pacemaker implantation may be needed for neonate. Therefore, it is very important to understand maternal and fetal condition and pay attention to the status of the neonate during delivery. In this paper, we present a case of patient with Sjogren's syndrome who underwent cesarean section under spinal anesthesia.

Neonatal lupus (NL) is a syndrome diagnosed in neonatal infants, whose mothers frequently suffer from autoimmune rheumatic diseases, manifested by two major signs: skin lesion and cardiac lesion. The paper gives data from the history of a description of the syndrome, as well as current ideas on its pathogenesis, considers both typical and rarer clinical manifestations of the disease, and presents approaches to NL therapy and prevention.

Background: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. Case Report: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin’s physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block. Conclusion: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants

Background: Complete Congenital Heart Block (CCHB) is a rare disease of the newborn that carries significant morbidity and mortality. CCHB can be diagnosed early or late in life. In newborns, it is usually associated with maternal autoantibodies or a congenital cardiac structural abnormality. The most common presentation of CCHB is bradycardia that can be diagnosed by an electrocardiogram. Results: This is a case report of a male infant born to a mother with an autoimmune disease, Systemic Lupus Erythematosus (SLE), who was found to have third degree heart block at birth. Conclusion: Early diagnosis and prompt management of the case is important for a better prog­nosis and prevention of associated complications. Neonates with CCHB should be managed at a tertiary care center and the only definite treatment is insertion of a pacemaker. Moreover, prenatal diagnosis and specific obstetric counseling of pregnant women with SLE along with careful monitoring with serial ultrasonography and echocardiography are of paramount importance in prevention of the disease in subsequent offspring.

Background: Fahr′s disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. No case of Fahr′s disease with associated cardiac conduction disease has been described in the literature to date. The objective of this case report was to describe a young female with various cardiac conduction system abnormalities and bilateral basal ganglia calcifica-tion suggestive of Fahr′s disease. Case Report: A 19-year-old female was transferred to our hospital for a pacemaker insertion. Her past medical history included cognitive impairment and asymptomatic congenital complete heart block since birth. Her manifestations in-cluded cognitive impairment, tremors, rigidity, ataxia, bilateral basal ganglia calcification without clinical manifesta-tions of mitochondrial cytopathy. She also had right bundle branch block, left anterior fascicular block, intermittent complete heart block, atrial arrhythmias with advanced atrioventricular blocks and ventricular asystole manifested by Stokes-Adams seizures, which was diagnosed as epilepsy. Conclusions: According to our knowledge, this was the first case report of a su spected association between Fahr′s disease and isolated cardiac conduction system disease. In addition, this case illustrated that in patients with heart blocks and seizures, a diagnosis of epilepsy needs to be made with caution and such patients need further evaluations by a cardiologist or electrophysiologist to consider pacing and prevent future catastrophic events.

A síndrome do lúpus neonatal (SLN) é uma doença auto-imune associada à presença de auto-anticorpos na circulação materno-fetal contra complexos ribonucléicos, SSA/Ro e SSB/La, e se caracteriza principalmente por bloqueio cardíaco congênito isolado (BCCI) e/ou manifestações cutâneas e hematológicas. A despeito da sua raridade, a SLN é a principal causa de BCCI, sendo responsável pela importante mortalidade (20% a 30%) e morbidade desses pacientes. A denominação de lúpus neonatal se baseia na semelhança das lesões cutâneas associadas à SLN nos neonatos com aquelas observadas em pacientes com lúpus eritematoso sistêmico (SLE). Por outro lado, o termo "isolado", para designar o BCC na SLN, é utilizado para especificar a ausência de malformações cardíacas congênitas e a ausência de infecções que causam alterações na condução átrio-ventricular (BAV). A SLN constitui-se num clássico modelo de auto-imunidade adquirida, no qual os anticorpos IgG maternos atravessam a barreira placentária e na circulação fetal podem exercer um papel importante na patogênese da síndrome. A presença quase universal dos anticorpos anti-Ro/SSA e anti-SSB/La no soro materno e fetal os inclui como marcadores para a SLN. Ao contrário da lesão cardíaca que compromete irreversivelmente a condução átrio-ventricular, os acometimentos cutâneos e/ou hematológicos são transitórios e podem regredir após o desaparecimento dos anticorpos maternos da circulação do lactente. Clinicamente, a SLN representa um desafio para profissionais reumatologistas, obstetras, neonatalogistas, dermatologistas e cardiologistas pediátricos que têm como meta identificar o risco gestacional de desenvolvimento da doença fetal, diagnosticar a síndrome precocemente e definir uma estratégia terapêutica adequada quando "in utero" ou pós-natal.Neonatal Lupus Syndrome (NLS) is an autoimmune disease associated to the presence of autoantibodies against ribonucleoproteins SSA/Ro and/or SSB/La in the maternal-fetal circulation and is characterized by isolated congenital heart block (ICHB) and/or cutaneous and hematological manifestations. Despite the rarity, NLS is the main cause of ICHB, which is responsible for the significant mortality (20%-30%) and morbidity of these patients. The neonatal lupus designation comes from the observation of cutaneous lesions similarities between NLS and systemic lupus erythematosus. The isolated term to define the CHB in NLS is employed to exclude cardiac abnormalities secondary to congenital structural malformations or infections. NLS is considered a model of passively acquired autoimmunity in which maternal autoantibodies cross the placenta and, once in fetal circulation, may play a role in the pathogenesis of the syndrome. The almost universal presence of anti-SSA/Ro and/or anti-SSB/La antibodies in the maternal and fetal circulation validates them as serological markers of NLS. Contrasting with the fetal cardiac tissue lesion, which is an irreversible process, cutaneous and hematological involvements are transitory manifestations and disappear after the clearance of maternal autoantibodies from the infant's circulation. It is important to notice that NLS represents a multidisciplinary challenge involving rheumatologists, obstetricians, neonatologists, dermatologists and pediatric cardiologists to identify the pregnancy at risk for NLS development and to introduce appropriate in utero or postnatal therapeutic strategies.

Anti-Ro/La negative congenital heart block (CHB) is uncommon. We report one such case of CHB, with no associated structural heart disease or maternal autoantibodies. The heart block reverted to sinus rhythm spontaneously at two weeks of age, and the patient remains in sinus rhythm at a one year followup. Whether patients with antibody negative complete heart block have a different clinical course is conjectural.

OBJETIVO: avaliar a freqüência de lúpus neonatal, prematuridade, natimortalidade, abortamento e baixo peso em gestações de pacientes com LES atendidas num serviço terciário. MÉTODOS: estudo retrospectivo dos atendimentos a pacientes com LES realizados no centro obstétrico do Hospital São Paulo/Unifesp/ EPM, entre novembro de 1991 e abril de 2003. Os dados clínicos e laboratoriais referentes à mãe e fetos foram obtidos através da revisão de prontuários médicos. RESULTADOS: foram identificadas 60 mulheres com LES que tiveram 75 gestações. A média de idade das mães por ocasião das gestações foi de 27,1± 6,1 anos e a mediana de tempo da doença foi de 48 meses. Duas gestantes necessitaram de diálise peritoneal. Sessenta e três pacientes fizeram uso de prednisona, 4 pacientes receberam pulsoterapia com solumedrol e 2 receberam azatioprina durante a gravidez. No primeiro trimestre da gestação, 6 pacientes haviam feito uso de difosfato de cloroquina e 2 utilizaram hidroxicloroquina. Uma paciente que havia recebido pulso de ciclofosfamida e outra que havia utilizado metotrexato desconhecendo a gravidez evoluíram com abortamento fetal espontâneo. Foram encontrados 13 óbitos intra-uterinos e 7 abordamentos. O parto foi prematuro em 57% das gestações e a média de tempo de gestação foi de 35 semanas. A média de peso ao nascer foi de 2.332±961g (variando de 525 a 3.620g). Foram detectados 5 casos de lúpus neonatal (8,9%), sendo um bloqueio cardíaco congênito (BCC) com óbito intrauterino na 29ª semana e 3 plaquetopenias associadas à anti-Ro/ SSA e 1 neutropenia associada ao anti-RNP. Com exceção do caso com BCC, estes anticorpos foram detectados no soro materno e no sangue do cordão umbilical. Ao final do estudo, foram detectados dois óbitos de recém-nascidos, com 9 e 130 dias de vida, em decorrência de complicações com a prematuridade. CONCLUSÕES: com relação à literatura, este estudo mostrou similar prevalência de perda fetal (aborto e óbito intra-uterino), entretanto, a freqüência de prematuridade foi maior que a média referida em outros trabalhos. Manifestações hematológicas do lúpus neonatal, como leucopenia e plaquetopenia foram observadas em freqüência maior do que a referida na literatura. Por serem transitórias e assintomáticas, estas alterações podem ter sido subdiagnosticadas.OBJECTIVE: to evaluate the frequency of neonatal lupus, prematurity, fetal loss, and low weight in pregnancies of SLE patients attended at a tertiary health service. MEHTODS: it is a retrospective study evaluating all SLE patients attended at obstetric center of Hospital São Paulo/Unifesp/EPM from November, 1991 to April, 2003. The mother and children's clinical and laboratory data were obtained reviewing medical records. RESULTS: sixty women and 75 pregnancies were identified. The mother age average during the pregnancies was 27.1±6.1 years old and the median of disease duration was 48 months. Two patients needed to be submitted to dialysis during the pregnancy. Sixty-three patients used prednisone, 4 received methilprednisolone pulsetherapy and 2 received azathioprine during the pregnancy. During the first trimester of pregnancy, 6 patients received diphosphate chloroquine and 2 received hidroxychloroquine. Two patients unknowing pregnancy received pulse of cyclophosphamide and another one had used methotrexate and both presented spontaneous abortion. It was observed 13 cases of intrauterine death and 7 abortions. Preterm birth occurred in 57% of pregnancies and the average duration of gestation was 35 weeks. The mean weight of the newborns was 2,332±961g (ranging from 525 to 3,620g). Five cases of neonatal lupus were identified (8,9%). One with congenital heart block (CHB) had intrauterine death at the 29th week of gestation, had 3 babies with thrombocytopenia associated with anti-Ro/SSA antibodies and one with neutropenia associated with anti-RNP antibody. Excepting the case of CHB with intrauterine death, these antibodies were detected on maternal and newborn blood sample. At the end of the study, it was detected two deaths of children with 9 and 130 days of life, due to complications related to prematurity. CONCLUSIONS: comparing with published reports this study showed a similar frequency of fetal loss (abortion and intrauterine death), however the frequency of prematurity was higher than the mean reported in the literature. Hematological features of neonatal lupus, as leucopenia and thrombocytopenia were observed at higher frequency than reported in the literature. As transitory and asymptomatic features, they could have been under-diagnosed.

Systemic lupus erythematosus is specially a disease of young women in their child bearing age. It has been found that SLE disease activity can repair in pregnancy even though patient is in complete remission state before the pregnancy. Besides this, pregnancy complications are higher in SLE patients. Another important issue is the use of drugs to control SLE because some of these drugs are potentially terotogenic. Fetal outcome is also a challenging issue. Therefore, multidisciplinary approach has key role in the management of Lupus pregnancy. Key words: Systemic lupus erythematosus (SLE); Lupus pregnancy; Lupus flare; Anti-phospholipid Antibody Syndrome; Neonatal Lupus Syndrome; Congenital heart block. DOI: 10.3329/bsmmuj.v3i1.5517 BSMMU J 2010; 3(1): 54-59