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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.

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.

Primary Sjögren's syndrome (pSS) is a condition that predominantly affects women. Reports of pregnancy outcome in these patients are limited and contradictory., Objective: To describe pregnancy characteristics and outcomes and newborn morbidity in women with pSS., Material and Methods: We included women with pSS who became pregnant after the onset of the symptoms of the disease. Clinical and serological characteristics, risk factors and previous maternal comorbidities are described. For each pregnancy in a woman with pSS, we recorded pregnancy course and outcome and newborn condition., Results: We assessed 11 patients with 18 pregnancies after the onset of pSS symptoms. All of them presented FAN +; 10 anti-Ro / SSA + and 7 anti-La / SSB +. The mean age in years at the onset of symptoms was 24.9 (SD 6.9) and at the time of pregnancy was 30.3 (SD 5.4). Thirteen pregnancies happened before the diagnosis, reporting only one miscarriage. Two preterm births, 1 case of oligohydramnios, 2 of premature membrane rupture and 2 low birthweight babies were reported after the onset of pSS symptoms. There was 1 newborn with congenital atrioventricular block and another with neonatal cutaneous lupus. All the women with pregnancy complications (n=6) had anti-Ro/SSA antibodies., Conclusions: Almost half of the pregnancies assessed in women with pSS were associated with complications not attributable to factors other than the disease., (Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Neonatal lupus erythematosus is an infrequent disease seen in newborns. It is caused by transplacental maternal autoantibody passage. Cutaneous involvement and congenital heart block (CHB) are the most common affections, although it may involve multiple organs like the liver, lungs, blood, nervous or digestive systems. This article present a review of the four cases diagnosed in the past five years in a Neonatal Unit, which shows the different clinical spectrum which can develop around this disease (CHB, multisystemic affection and two cutaneous cases), different autoantibodies (specially anti-SSA) with an early negativization during the first year of life and the possibility of future collagen vascular disease as occurred in one case., (Copyright © 2013 Elsevier España, S.L. All rights reserved.)