Neonatal lupus (NL), a passively-acquired autoimmune disease associated with maternal anti-SSA antibody, presents both cardiac manifestations such as cardiac NL and non-cardiac manifestations including rashes, cytopenia, and hepatic abnormalities. Cardiac NL, occurring in 1-2% of anti-SS-A antibody-positive mothers, is a life-threatening complication with a mortality rate of 20% and a pacemaker implantation rate of 70%. In contrast, cutaneous NL, which is more common than cardiac NL, usually resolves in six months. Read More
Objective: Recent studies have suggested that prenatal exposure to HCQ reduces the risk of cardiac neonatal lupus. The aim of this study is to assess if maternal intake of antimalarials (AMs) throughout pregnancy lowered the risk of cardiac and non-cardiac neonatal lupus.
Methods: Consecutive children seen between 1 January 1984 to 1 October 2013 born to women with a CTD and positive anti-Ro and/or anti-La antibodies were eligible for this single-centre retrospective cohort study. Read More
Neonatal lupus erythematosus is an autoimmune disease resulting from the trans-placental passage of maternal anti-SSA/Ro, anti-SSB/La, and less frequently anti-RNP antibodies to the foetus. At the time of diagnosis 50% of mothers are asymptomatic. Neonatal manifestations of this multisystem disease may include congenital heart block, cutaneous lesions and haematological abnormalities. Read More
A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Read More
Objectives: Autoimmune congenital heart block (CHB) is associated with placental transcytosis of maternal autoantibodies directed against Ro/SS-A and La/SS-B. However, only about 2% of children born to mothers with the respective antibodies are affected, indicating that further risk factors exist, which are not yet fully understood. In this study, we investigated whether a maternal type I interferon (IFN) signature represents a risk factor for the development of CHB. Read More
Rheum Dis Clin North Am 2017 May 14;43(2):215-226. Epub 2017 Mar 14.
Division of Rheumatology, Johns Hopkins Lupus Center, Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 7500, Baltimore, MD 21205, USA. Electronic address:
Systemic lupus erythematosus (SLE) is an autoimmune disease with a strong female predilection. Pregnancy remains a commonly encountered but high-risk situation in this setting. Both maternal and fetal mortality and morbidity are still significantly increased despite improvements in outcomes. Read More
Introduction. Neonatal lupus erythematosus (NLE) is an acquired disease of the newborn caused by transplacental transfer of maternal anti-Ro/SSA, anti-La/SSB, and infrequently anti-U1 RNP antibodies. Methodology. Read More
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal-neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Read More
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur. Read More
We report a case of systemic lupus erythematosus (SLE) in a young woman who became pregnant amid a severe flare. She continued to have active disease in the face of aggressive treatments complicated by several side effects of immunosuppressive drugs including recurrent sepsis and gestational diabetes. Her fetus was at risk for congenital heart block during the second and third trimesters. Read More
Obstetric and Gynecology Department, High Risk Pregnancy Unit, Autoimmune diseases and pregnancy clinic, University Hospital La Paz, Paseo de la Castella 261, 28046, Madrid, Spain.
Anti-Ro/SS-A is one specific type of antinuclear antibodies. They are in the majority of cases associated with primary Sjögren syndrome (SS) but also in Systemic Lupus Erythematosus (SLE), rheumatoid arthritis (RA), and in healthy people. During pregnancy, they are mainly associated to congenital heart block (CHB) and neonatal lupus (NL). Read More
In Down syndrome, so far, has not been reported it's association with congenital heart block, this entity is rare and occurred in only 1% of mothers who have systemic lupus erythematosus, the presence of anti-Ro antibodies cross the placenta presenting neonatal lupus with skin lesions and congenital heart block, bradycardia, which is why we describe the following case. This is a new male end product of asymptomatic young mother, but serological birth with stigmata of Down syndrome, birth presents congenital bradycardia rare manifestation abnormalities, but common in children of mothers with lupus are initiated study protocol, realizing you including laboratory tests and immunological studies cabinet as electrocardiogram and echocardiogram, which gave tone to take the mother immunological studies, being positive antiRo. We concluded that the risk of heart block in a patient with Down syndrome is the same as for the general population. Read More
Objectives: Autoimmune diseases do not impair fertility, and women with autoimmune diseases who become pregnant are likely to experience more complicated pregnancies than are women without the disease. Pregnancies complicated by these disorders have a high clinical impact on both the pregnancy and the disease. The effect of autoimmune disease on pregnancy differs according to the type of maternal disease, disease activity, severity of organ damage, antibody profile, and drug treatment. Read More
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Read More
Neonatal lupus erythematosus is an autoimmune disorder mainly affecting the heart and skin. It is the most common cause of congenital heart block. In addition, hematological, hepatobiliary and neurological involvement may occur. Read More
Objective: To investigate the clinical characteristics and adverse pregnancy outcomes in pregnant women with new onset systemic lupus erythematosus (SLE) during pregnancy. Methods: The clinical data of 263 pregnancies with SLE in the First Affiliated Hospital of Zhongshan University from 2001 to 2015 were collected and analyzed retrospectively. Results: Of all the 263 pregnancies, 188 were diagnosed before pregnancy and 75 were newly diagnosed during pregnancy. Read More
While apoptotic debris is believed to constitute the original antigenic insult in lupus (which is characterized by a time-dependent diversification of autoreactivity), whether such debris and autoantibodies specifically recognizing its constituents mediate differential effects on innate and humoral responses in lupus-prone mice is currently unknown. Apoptotic blebs (as opposed to cellular lysate) enhanced preferentially the maturation of dendritic cells (DCs) from bone marrow precursors drawn from lupus-prone mice. Murine, somatically mutated, apoptotic cell-reactive immunoglobulin (Ig)G monoclonal antibodies demonstrated enhanced recognition of DCs and also displayed a prominent lupus strain-specific bias in mediating DC maturation. Read More
J Autoimmun 2016 Nov 2;74:106-117. Epub 2016 Jul 2.
Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Italy; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. Electronic address:
Nowadays, most of the young women affected by Systemic Lupus Erythematosus (SLE) can carry out one or more pregnancies thanks to the improvement in treatment and the consequent reduction in morbidity and mortality. Pregnancy outcome in these women has also greatly improved in the last decades. A correct timing for pregnancy (tailored on disease activity and established during a preconception counselling), together with a tight monitoring during the three trimesters and the post-partum period (to timely identify and treat possible obstetric complications or maternal disease flares), as well as the concept of multidisciplinary management, are currently milestones of the management of pregnancy in SLE patients. Read More
Systemic lupus erythematosus (SLE) mostly affects young women of reproductive age group. SLE patients may conceive as any normal woman but complication may occur in these patients if the disease is active. Pregnancy in SLE may lead to 1. Read More
Reproductive issues including contraception, fertility, and pregnancy are important components of the comprehensive care of women with systemic lupus erythematosus (SLE). SLE pregnancies are complicated due to risk for maternal disease exacerbation and potential for fetal and neonatal complications. Pre-pregnancy assessment is important to identify patients with severe disease-related damage who should avoid pregnancy, counsel patients to conceive when disease has been stable and inactive on appropriate medications, and assess relevant risk factors including renal disease, antiphospholipid antibody, and anti-Ro/SS-A and anti-La/SS-B antibodies. Read More
Background: Autoimmune fetal congenital heart block (CHB) is the most severe manifestation of neonatal lupus, and it is seen when maternal autoimmune antibodies cross the placenta and damage the AV node of the fetus. CHB is mainly associated with maternal SLE with anti-Ro/SSA- and anti-La/SSB-positive status, and incidence of CHB increases when both the antibodies are present. This study was conducted to know the incidence of fetal CHB in patients of SLE who had ANA, anti-Ro/SSA and anti-La/SSB positivity. Read More
Department of Medicine and Therapeutics, Korle-bu Teaching Hospital, P.O. Box KB 77 Accra, Ghana; School of Medicine and Dentistry, College of Health Sciences, University of Ghana P.O. Box GP 4236, Accra.
Congenital heart block (CHB) is a potentially lethal condition characterized by a third-degree atrioventricular block (AVB). Despite anti-Ro52 antibodies being detected in nearly 90% of mothers of affected children, CHB occurs in only 1-2% of anti-Ro/Sjögren's-syndrome-related antigen A (SSA) autoantibody-positive pregnancies. Maternal antibodies have been suggested to bind molecules crucial to fetal cardiac function; however, it remains unknown whether a single antibody profile associates with CHB or whether several specificities and cross-reactive targets exist. Read More
Neonatal lupus erythematosus (NLE) affects 1%-2% pregnant females with autoimmunity. An infant presented with steroid refractory hemolytic anemia as a manifestation of NLE. A trial of withholding breastfeeding had a transient response, but infant was eventually put on cyclosporin therapy to control the hemolysis. Read More
A 3-day-old girl was referred from her pediatrician for oral ulcerations. The patient was otherwise well appearing and afebrile. Her prenatal and antenatal courses were unremarkable, except for a failed routine hearing screen. Read More
Background: Children from mothers with systemic lupus erythematosus are frequently born with congenital heart block. This study aimed at evaluating long-term outcome because long-term data are scarce.
Methods: In the database of pediatric and congenital heart disease (University Hospitals Leuven), 19 children from systemic lupus erythematosus mothers and who were born with or developed atrioventricular block were identified. Read More
We report a case of pathological foetal Doppler velocity, specifically the absence of end diastolic flow in the umbilical artery (AEDV/REDV), suspected diabetic pregnancy and mesangioproliferative glomerulonephritis, at 32 weeks of gestation. The foetal heart rate tracings were evaluated using a computerised cardiotocogram (Oxford Sonicaid system 8002 Chichester, England) 1 for 20-30 min parallel to the routine cardiotocogram. The ultrasound control at 33 weeks of gestation showed oligohydramnion, foetal centralisation and reduced interval foetal growth. Read More
Neonatal lupus erythematous (NLE) is a rare autoimmune disease caused by placental transfer of maternal anti-SSA/Ro or anti-SSB/La antibodies. It usually presents with transient cutaneous lesions, congenital heart block and other systemic symptoms. The authors report a case of neonatal lupus erythematosus who presented with targetoid-like lesions on both feet. Read More
Ann Dermatol Venereol 2016 Oct 26;143(10):590-600. Epub 2016 Apr 26.
Department of internal medicine, medical center, reference center for rare systemic and autoimmune diseases, hôpital Cochin, AP-HP, université René-Descartes, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France. Electronic address:
A causal link has long been described between estrogen and systemic lupus erythematosus activity. Contraceptive and pregnancy management is now common for lupus patients, but pregnancy continues to be associated with higher maternal and fetal mortality/morbidity in systemic lupus erythematosus patients than among the general population. Potential complications include lupus flares, obstetric complications (fetal loss, in utero growth retardation, premature birth) and neonatal lupus syndrome. Read More
Background: Neonatal lupus erythematosus (NLE) is not a common disease. The death rate of complete congenital heart block (CCHB), which is the most severe clinical manifestation, is as high as 20% to 30%, so early recognition of infants at risk is important.
Objectives: To investigate the clinical features and long-term prognosis of NLE. Read More
Background/objectives: The manifestations of cutaneous lupus erythematosus (CLE) and their relevance to systemic disease are well characterized in adults, but data are limited in children. The objective of the current study was to examine the spectrum of CLE and its relationship to systemic disease in children from a tertiary care pediatric dermatology clinic.
Materials And Methods: An analysis of 26 children with CLE registered consecutively over 14 years was performed. Read More
This report describes the case of a very low-birth-weight male infant with neonatal lupus erythematosus. His mother had Sjögren's syndrome, and her previous child had suffered a complete heart block. Accordingly, maternal steroid (betamethasone) therapy was administered to prevent a congenital heart block for 15 weeks (from 13 to 27 weeks' gestation). Read More
Objectives: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit. Read More
Background: Neonatal Lupus Erythematosus (NLE) is an uncommon autoimmune disease characterized by cutaneous, hepatic, hematological, neurological and cardiac involvement.
Case Presentation: Here we report four cases of cutaneous NLE which were referred to our department in the last 10 years and update literature. The newborns presented with different skin, clinical and laboratory features. Read More
We studied the cytokine profile of two siblings with neonatal lupus erythematosus (NLE) born to a mother positive for serum anti-Ro and -La antibodies, who did not receive any medication during the two pregnancies. The first sibling was found to have complete atrioventricular block in utero and became severely ill after birth. He fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis on day 2. Read More
Toll-like receptors (TLRs), first identified as pattern recognition receptors, are now recognized to serve as a key interface between innate and adaptive immunity. Systemic lupus erythematosus (SLE) is characterized by both continuous and cyclic stimulation of the innate and adaptive immune system by endogenous nucleic acids released from apoptotic or necrotic cells. TLR7 and TLR9 function as innate sensors of viral infection as their ligands are ssRNA and dsDNA, respectively. Read More
Introduction: Neonatal lupus syndrome (NLS) is a passively acquired autoimmune condition due to the transplacental passage of maternal anti-Ro/SSA and anti-La/SSB antibodies in mothers with systemic lupus erythematosus (SLE), and congenital complete heart block (CHB) is its most serious manifestation. Skin and hepatic involvement may occur in later infancy.
Case Presentation: A term infant with fetal bradycardia, detected at the 23rd gestational age, was diagnosed with CHB due to NLS and was successfully treated with a permanent epicardial pacemaker. Read More
Objective: To determine the maternal predictive factors for fetal congenital heart block (CHB) in pregnancy in mothers positive for anti-SS-A antibodies.
Methods: The Research Team for Surveillance of Autoantibody-Exposed Fetuses and Treatment of Neonatal Lupus Erythematosus, the Research Program of the Japan Ministry of Health, Labor and Welfare, performed a national survey on pregnancy of mothers positive for anti-SS-A antibodies. We analyzed 635 pregnant mothers who tested positive for anti-SS-A antibodies before conception but had no previous history of fetal CHB. Read More
The aims of our study are to observe the pregnancy outcome of anti-Sjögren's syndrome-related antigen A (SSA)/Ro-positive women and to predict the risk factors for adverse pregnancy outcome and neonatal lupus erythematosus (NLE). Clinical data of 126 anti-SSA/Ro-positive patients with 140 pregnancies were evaluated retrospectively, and the newborns were followed up as a cohort in 3 months. χ (2) test or logistic regression was used to predict the risk factors of lupus flares during pregnancy, fetal loss, and NLE. Read More
Spedali Civili di Brescia, Brescia, Italy (Dr Taraborelli); University of Brescia, Brescia, Italy (Dr Taraborelli); University of Pavia, Pavia, Italy (Dr Taraborelli); Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, New York, New York (Drs Erkan and Taraborelli); Weill Medical College, Cornell University, New York, New York (Dr Erkan). Mara Taraborelli, MD, is a rheumatology fellow at the Spedali Civili di Brescia and the University of Brescia in Italy. She is also affiliated with the University of Pavia in Italy and the Hosptial for Special Surgery in New York City. Doruk Erkan, MD, MPH, is an associate physician-scientist at the Barbara Volcker Center for Women and Rheumatic Diseases and an associate attending physician at the Hospital for Special Surgery in New York City, where he is also an associate professor of medicine at Weill Medical College of Cornell University.
The awareness of pregnancy-related physiologic changes and complications is critical for the appropriate assessment and management of pregnant patients with systemic autoimmune diseases. The overlapping features of physiologic and pathological changes, selected autoantibodies, and the use of potentially teratogenic medications can complicate their management during pregnancy. While pregnancy in lupus patients presents an additional risk to an already complex situation, in patients with no disease activity, the risk of a future pregnancy-related complication is relatively low. Read More
Background: Cardiac manifestations of neonatal lupus (cardiac NL) include congenital heart block and cardiomyopathy. Several candidate biomarkers were evaluated in cases at risk for cardiac NL on the basis of potential roles in inflammation, fibrosis, and cardiac dysfunction: C-reactive protein (CRP); NT-pro-B-type natriuretic peptide (NT-proBNP); troponin I; matrix metalloproteinase (MMP)-2; urokinase plasminogen activator (uPA); urokinase plasminogen activator receptor (uPAR); plasminogen; and vitamin D.
Objectives: Identification of maternal and fetal biomarkers associated with development and morbidity of cardiac NL should provide clues to pathogenesis with translational implications for management. Read More
Autoimmun Rev 2015 Dec 15;14(12):1154-60. Epub 2015 Aug 15.
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. Electronic address:
Background: Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB. Read More
The authors reported histiocytoid neutrophilic dermatitis in neonatal lupus erythematosus (NLE). One-month-old male infant presented with annular erythematous plaques at the face and trunk. Serologic studies revealed positive anti-ribonuclear protein antibodies (RNP) and antinuclear antibodies (ANA). Read More
Systemic lupus erythematosus (SLE) is a chronic relapsing-remitting autoimmune disease affecting several organs. Although the management of lupus patients has improved in the last years, several aspects still remain challenging. More sensitive and specific biomarkers for an early diagnosis as well as for monitoring disease activity and tissue damage are needed. Read More