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BMJ Case Rep 2020 Jun 11;13(6). Epub 2020 Jun 11.

Department of Pediatrics and Neonatology, Cloudnine Hospital, Gurgaon, Haryana, India.

Neonatal lupus erythematosus (NLE) should be considered when a newborn develops atrioventricular heart block along with the presence of autoantibodies to Sjogren's syndrome autoantigens in the maternal serum. NLE can also present with features such as cutaneous lesions, hepatic dysfunction or haematological abnormalities. Differential diagnosis usually includes congenital infections as there is a significant overlap of symptoms with NLE. Read More

Front Cardiovasc Med 2020 7;7:83. Epub 2020 May 7.

AP-HP, Cochin Hospital, Internal Medicine Department, Referral center for Rare Autoimmune and Systemic Diseases, Paris, France.

Arch Dis Child Fetal Neonatal Ed 2020 Jul 3;105(4):356. Epub 2020 May 3.

Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Emilia-Romagna, Italy.

Prenat Diagn 2020 Apr 13. Epub 2020 Apr 13.

Division of Maternal Fetal Medicine, Department of Obstetrics & Gynecology, NYU Langone Medical Center, New York, New York, USA.

Systemic lupus erythematosus (SLE) primarily affects women of childbearing age and is commonly seen in pregnancy. The physiologic and immunologic changes of pregnancy may alter the course of SLE and impact maternal, fetal, and neonatal health. Multidisciplinary counseling before and during pregnancy from rheumatology, maternal fetal medicine, obstetrics, and pediatric cardiology is critical. Read More

Ann Dermatol Venereol 2020 Apr 12;147(4):258-264. Epub 2020 Mar 12.

Service de dermatologie, hôpital Robert-Debré, 75019 Paris, France.

Introduction: Neonatal lupus erythematosus (NEL) is a rare condition secondary to transplacental transfer of maternal anti-nuclear antibodies, generally anti-Ro/SSA. The most common signs are dermatological and cardiac. The most frequently reported clinical association is periorbital erythema, known as "owl eye", and bipolar erythematous maculopapular plaques with fine scales. Read More

Pediatr Dermatol 2020 May 28;37(3):484-489. Epub 2020 Feb 28.

Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Background: Neonatal lupus erythematosus (NLE) results from cross-placental transfer of maternal autoantibodies. Neonates can present with cardiac, cutaneous, hepatobiliary, hematologic, and neurologic complications from antibody-mediated organ toxicity. Scant evidence exists on long-term clinical characteristics and outcomes of patients with neonatal lupus. Read More

CMAJ 2020 Feb;192(7):E163

Department of Dermatology (Liu, Ma), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

Arch Dis Child 2020 Feb 7. Epub 2020 Feb 7.

Department of Paediatrics/Paediatric Rheumatology, University Hospitals of Leicester NHS Trust, Leicester, UK.

Turk J Med Sci 2020 01 9;50(1):281-290. Epub 2020 Jan 9.

Division of Rheumatology, Department of Internal Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey

Background/aim: Neonatal lupus erythematosus (NLE) is an autoimmune syndrome caused by transplacental transmission of maternal autoantibodies, often with devastating consequences. The objective of this systematic literature review was to analyze the demographic data, geoepidemiology, clinical, and serological characteristics associated with NLE.

Materials And Methods: We performed a systematic literature search of the Pubmed database covering the period from 1976 to August 2015, using the MeSH terms “neonatal lupus” or “congenital heart block”. Read More

Am J Perinatol 2020 Jul 3;37(9):873-880. Epub 2020 Jan 3.

Division of Epidemiology, Department of Health Research and Policy, Stanford University School of Medicine, Stanford, California.

Objective:  Systemic lupus erythematosus (SLE) increases the risk of complications in pregnancy. Hydroxychloroquine (HCQ) decreases flares and neonatal lupus syndrome. Limited evidence suggests that HCQ also reduces preeclampsia and preterm birth in SLE pregnancies. Read More

Front Pediatr 2019 20;7:465. Epub 2019 Nov 20.

School of Medicine, Shandong University, Jinan, China.

To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases. 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. In total, 16 patients were identified, including 2 fetuses, and 14 children. Read More

Best Pract Res Clin Obstet Gynaecol 2020 Apr 23;64:85-96. Epub 2019 Oct 23.

Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA. Electronic address:

Assisted reproductive technology (ART) procedures are safe for women with rheumatic autoimmune diseases (rAID) when illness is inactive. Medications incompatible with pregnancy should be replaced with alternative pregnancy-compatible medications months before planned ART procedures to allow time to verify the substitute medication's efficacy and tolerability. Medications compatible with pregnancy should be continued, as should anticoagulation (warfarin changed to low-molecular-weight heparin) before pregnancy begins. Read More

J Autoimmun 2020 Feb 31;107:102354. Epub 2019 Oct 31.

Department of Biostatistics and Data Science, Wake Forest School of Medicine, Winston-Salem, NC, USA.

Mothers giving birth to children with manifestations of neonatal lupus (NL) represent a unique population at risk for the development of clinically evident pathologic autoimmunity since many are asymptomatic and only become aware of anti-SSA/Ro positivity (anti-Ro+) based on heart block in their fetus. Accordingly, we hypothesized that the microbiome in saliva is associated with the development of autoreactivity and in some cases the progression in health status from benign to overt clinical disease including Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE). The study comprised a clinical spectrum of anti-Ro+ mothers, all of whom gave birth to a child with NL: 9 were asymptomatic or had an undifferentiated autoimmune disease (Asym/UAS) and 16 fulfilled criteria for SS and/or SLE. Read More

Ann Rheum Dis 2020 02 31;79(2):217-224. Epub 2019 Oct 31.

Medicine, NYU School of Medicine, New York, New York, United States.

Objectives: Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality; however, there is minimal information on long-term outcomes of affected individuals. This study was initiated to evaluate the presence of and the risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counselling, to further understand pathogenesis and to provide potential preventative strategies.

Methods: Echocardiogram reports were evaluated in 239 individuals with cardiac NL: 143 from age 0-1 year, 176 from age >1-17 years and 64 from age >17 years. Read More

J Am Acad Dermatol 2019 Oct 15. Epub 2019 Oct 15.

Department of Medicine, University of Toronto, Toronto, Ontario, Canada; Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.

Background: Cutaneous eruptions in neonatal lupus erythematosus (NLE) are thought to be self-resolving. Limited literature suggests cutaneous changes may persist.

Objective: To characterize cutaneous residua in NLE and identify predictors for their development. Read More

Indian Pediatr 2019 08;56(8):701-702

Department of Dermatology and STD, Lady Hardinge Medical College, New Delhi, India.

Best Pract Res Clin Rheumatol 2018 12 15;32(6):750-766. Epub 2019 Apr 15.

Dr. Morris Ziff Distinguished Professorship in Rheumatology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA. Electronic address:

Management of pregnancy in patients with rheumatic and musculoskeletal disorders is both challenging and rewarding. Most patients with rheumatic disease can have a successful pregnancy. Pregnancy outcome is improved when maternal autoimmune disease is stable or quiescent. Read More

Arch Dis Child Fetal Neonatal Ed 2019 Nov 30;104(6):F630. Epub 2019 Jul 30.

Department of Dermatology, Postgraduate Institute of Medical Education and Research and Dr Ram Manohar Lohia Hospital, New Delhi, India.

Trends Cardiovasc Med 2020 Jul 20;30(5):275-286. Epub 2019 Jun 20.

Third Department of Cardiology, Athens University School of Medicine, Athens, Greece. Electronic address:

Congenital complete heart block (CCHB) occurs in 2-5% of pregnancies with positive anti-Ro/SSA and/or anti-La/SSB antibodies, and has a recurrence rate of 12-25% in a subsequent pregnancy. After trans-placental passage, these autoantibodies attack and destroy the atrioventricular (AV) node in susceptible fetuses with the highest-risk period observed between 16 and 28 weeks' gestational age. Many mothers are asymptomatic carriers, while <1/3 have a preexisting diagnosis of a rheumatic disease. Read More

Neoreviews 2019 05;20(5):e298-e301

Neonatal Medicine and.

Lupus 2019 Jun 21;28(7):888-892. Epub 2019 May 21.

Department of Dermatology, Gachon Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.

The clinical characteristics of cutaneous lupus erythematosus (CLE) are well delineated in adults, but pediatric data, particularly in Asian populations, are limited. Therefore, we evaluated the characteristics of pediatric cases by retrospectively reviewing the medical records of children with CLE during a 15-year period in a tertiary care dermatology clinic in South Korea. The study included 21 children (8 males and 13 females), 4 of whom had neonatal lupus erythematosus (NLE). Read More

Crit Rev Biochem Mol Biol 2019 04 14;54(2):133-152. Epub 2019 May 14.

a RNA Biology Laboratory, Center for Cancer Research , National Cancer Institute , Frederick , MD , USA.

Ro60, also known as SS-A or TROVE2, is an evolutionarily conserved RNA-binding protein that is found in most animal cells, approximately 5% of sequenced prokaryotic genomes and some archaea. Ro60 is present in cells as both a free protein and as a component of a ribonucleoprotein complex, where its best-known partners are members of a class of noncoding RNAs called Y RNAs. Structural and biochemical analyses have revealed that Ro60 is a ring-shaped protein that binds Y RNAs on its outer surface. Read More

Rheumatology (Oxford) 2019 11;58(11):2000-2008

Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences and SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy.

Objectives: In this study we aimed to investigate foetal and maternal pregnancy outcomes from a large multicentre cohort of women diagnosed with MCTD and anti-U1RNP antibodies.

Methods: This multicentre retrospective cohort study describes the outcomes of 203 pregnancies in 94 consecutive women ever pregnant who fulfilled the established criteria for MCTD with confirmed U1RNP positivity.

Results: The foetal outcomes in 203 pregnancies were as follows: 146 (71. Read More

Lupus 2019 May 1;28(6):783-785. Epub 2019 May 1.

5 Department of Neonatology, Al Hada Armed Forces Hospital, KSA.

Neonatal thrombosis is considered a rare manifestation with unclear aetiology. We reported a neonatal lupus of a Sjogren's syndrome mother with recurrent miscarriage secondary to antiphospholipid syndrome; seronegative to anticardiolipin antibodies, lupus anticoagulant and B2GP1. She was serologically positive to antiphosphatidylethanolamine and antiprothrombin antibodies, anti-SSA/Ro and anti-SSB/La. Read More

Pan Afr Med J 2018 17;31:117. Epub 2018 Oct 17.

Faculté de Médecine et d'Odonstomatologie Usttb, Bamako, Mali.

Neonatal lupus is rare. It is due to the transmission of maternal autoantibodies across the placenta during pregnancy. We here report the case of a 2-month old female infant treated for erythematous macular cutaneous lesions on the face and the trunk. Read More

J Obstet Gynaecol India 2019 Apr 12;69(2):104-109. Epub 2019 Mar 12.

The Department of Obstetrics and Gynecology, Nowrosjee Wadia Maternity Hospital, Mumbai, India.

Systemic lupus erythematosus is a chronic multisystemic autoimmune disease that predominantly affects young women of childbearing age group. There is a complex immunologic interplay during pregnancy in patients with systemic lupus erythematosus. The pregnancy has direct impact on the disease where an increased rate of flares is noted, and lupus leads to increased risk of hypertensive diseases of pregnancy, preterm birth as well as miscarriages, particularly those with antiphospholipid antibodies. Read More

Pediatr Rev 2019 Apr;40(4):e14-e17

Department of Dermatology, University of Cincinnati Medical Center, Cincinnati, OH.

Obstet Med 2019 Mar 19;12(1):31-37. Epub 2018 Sep 19.

Internal Medicine Division, Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, QC, Canada.

Objective: To investigate the impact of medical and obstetric complications associated with mixed connective tissue disease (MCTD) in pregnancy.

Method: We analyzed 68 pregnancies from a systematic literature review and 12 pregnancies affected by MCTD at our centre between 1986 and 2015 for medical and obstetric complications.

Results: During pregnancy 37. Read More

Skinmed 2019 19;17(1):76-78. Epub 2019 Mar 19.

Lady Hardinge Medical College & Smt. S. K. Hospital, New Delhi, India.

A preterm, low birth weight (1.45 kg) baby boy, delivered at 32 weeks, was admitted to the neonatal ICU with a low APGAR score assessment of Activity/muscle tone, Pulse/heart rate, Grimace, Appearance, and Respiration. Each criterion is graded from 0 to 2, and a final score is obtained by their sum (0-10). Read More

Int J Pediatr Adolesc Med 2018 Dec 13;5(4):127-130. Epub 2018 Dec 13.

Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

Background: Conduction disturbances other than heart block related to neonatal lupus are rarely explored and reported in children with systemic lupus erythematosus (SLE).

Objective: To report the electrocardiographic (ECG) abnormalities in children with SLE and assess whether anti-Ro/SSA antibodies and hydroxychloroquine are associated with the rhythm disturbances.

Methods: This cross-sectional retrospective study comprised patients with SLE who had regular follow-up in the Pediatric Lupus Clinic at King Faisal Specialist Hospital and Research Center-Riyadh. Read More

Curr Opin Obstet Gynecol 2019 04;31(2):120-126

Stanford University, Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Stanford, California, USA.

Purpose Of Review: The aim of this study was to describe risks of systemic lupus erythematosus (SLE) in pregnancy and the importance of preconception counselling, medication optimization and close surveillance.

Recent Findings: Advances in care for pregnant patients with SLE have led to improved obstetric outcomes, but maternal and foetal risks continue to be elevated. Conception during periods of disease quiescence and continuation of most medications decrease adverse pregnancy outcomes. Read More

Ultrasound Obstet Gynecol 2019 Jul 7;54(1):87-95. Epub 2019 Jun 7.

Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.

Objectives: Assuming that autoimmune congenital heart block (CHB) is a progressive disease amenable to therapeutic modulation, we introduced a surveillance program for at-risk pregnancies with the dual aim of investigating if fetal atrioventricular block (AVB) could be detected and treated before becoming complete and irreversible, and to establish the incidence of AVB I, II and III in a large prospective cohort.

Methods: This was a prospective study of 212 anti-Ro52 antibody-exposed pregnancies at risk of fetal AVB that were followed weekly between 18 and 24 weeks' gestation at our tertiary fetal cardiology center from 2000 to 2015. A 12-lead electrocardiogram (ECG) was recorded within 1 week after birth. Read More

Arch Dis Child Fetal Neonatal Ed 2019 Mar 22;104(2):F191. Epub 2018 Dec 22.

Department of Neonatology and Pediatrics, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain.

Mediterr J Rheumatol 2018 Dec 18;29(4):228-231. Epub 2018 Dec 18.

Rheumatology and Clinical Immunology and Allergiology Clinic, PAGNI, Herakleion, Crete, Greece.

Pregnancy in women with SLE (Systematic Lupus Erythematosus) is considered of "high risk" since it has been related with adverse events both in the mother and the foetus. Many studies have reported relapse of the disease during the pregnancy and the first trimester post-labour, while others have found no difference in terms of frequency and type of relapses. Moreover, adverse obstetrical events like recurrent pregnancy loss, preeclampsia, prematurity, intrauterine growth restriction and neonatal lupus syndrome tend to occur more often in patents with SLE. Read More

Congenit Heart Dis 2019 Mar 16;14(2):221-229. Epub 2018 Nov 16.

Children's Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Objective: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization. Read More

J Cutan Pathol 2019 Mar 26;46(3):216-220. Epub 2018 Dec 26.

Department of Pathology, Duke University, Durham, North Carolina.

Neutrophilic figurate erythema of infancy (NFEI) is a rare variant of annular erythema of infancy. It is characterized by annular erythematous plaques, occasionally with a polycyclic configuration. The main challenge is to differentiate this rare entity from other figurate erythemas associated with serious diseases such as neonatal lupus erythematosus. Read More

Acta Reumatol Port 2018 Jul-Sep;43(3):235-236

Hospital de Santa Maria.

A 24-day-old female newborn presented with multiple annular erythematous plaques with a targetoid shape, on the forehead, neck and upper trunk, that had begun ten days earlier. She had been diagnosed with citrullinemia type 1 in the first week of life and arginine supplementation was initiated. Her personal and family history was otherwise unremarkable. Read More

Acta Reumatol Port 2018 Jul-Sep;43(3):230-234

Neonatal Intensive Care Unit.

Introduction: Pulmonary involvement is relatively frequent in adult and juvenile patients with Systemic Lupus Erythematosus (SLE), but its occurrence in newborns with Neonatal Lupus Erythematosus (NLE) is exceedingly rare.

Case Report: A mother with SLE and positive anti-SSA/Ro and anti-SSB/La delivered a preterm newborn with third-degree heart block and positive anti-SSA/Ro confirmed postnatally. A temporary pacemaker was placed at D3 and a definitive pacemaker only at D15 due to sepsis with concurrent mild respiratory failure. Read More

Medicine (Baltimore) 2018 Nov;97(45):e13185

Department of Pediatrics, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Rationale: Neonatal lupus erythematosus (NLE) is an infrequent disease caused by transplacental maternal autoantibodies. The most common effects of NLE include cutaneous involvement and congenital heart block (CHB), although it might involve multiple organs, such as the liver, lungs, blood, and nervous or digestive systems. Izmirly PM1 and Tonello et al recently reported cutaneous manifestations of neonatal lupus and risk of subsequent CHB. Read More

J Am Coll Cardiol 2018 10;72(16):1940-1951

Division of Cardiology, Department of Pediatrics, Hospital for Sick Children, Toronto, Canada.

Background: Fetal atrioventricular block (AVB) occurs in 2% to 4% of anti-Ro antibody-positive pregnancies and can develop in <24 h. Only rarely has standard fetal heart rate surveillance detected AVB in time for effective treatment.

Objectives: Outcome of anti-Ro pregnancies was surveilled with twice-daily home fetal heart rate and rhythm monitoring (FHRM) and surveillance echocardiography. Read More

Ann Rheum Dis 2018 12 8;77(12):1742-1749. Epub 2018 Oct 8.

Division of Rheumatology, New York University School of Medicine, New York City, New York, USA.

Objective: Cutaneous neonatal lupus (cNL) occurs in possibly 5%-16% of anti-Ro±anti-La antibody-exposed infants. Data suggest in utero exposure to hydroxychloroquine (HCQ) may prevent cardiac NL. The aim was to assess whether in utero exposure to HCQ decreases the risk of cNL and/or delays onset. Read More

Lupus 2018 12 3;27(14):2298-2299. Epub 2018 Oct 3.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

JAAD Case Rep 2018 Sep 14;4(8):780-783. Epub 2018 Sep 14.

National Health Laboratory Services, Red Cross Children's Hospital, University of Cape Town, Cape Town, South Africa.

Rheumatology (Oxford) 2018 07;57(suppl_5):v9-v17

Division of Rheumatology, New York University School of Medicine, NY, USA.

Objective: To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies.

Methods: A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy. Read More

J Cutan Pathol 2018 Oct 19;45(10):791-793. Epub 2018 Jul 19.

Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.

Rev Bras Ginecol Obstet 2018 Apr 27;40(4):209-224. Epub 2018 Apr 27.

Department of Obstetrics and Gynecology, Universidade Estadual de Campinas (Unicamp), Campinas, SP, Brazil.

Objective:  To review the existing recommendations on the prenatal care of women with systemic lupus erythematosus (SLE), based on currently available scientific evidence.

Methods:  An integrative review was performed by two independent researchers, based on the literature available in the MEDLINE (via PubMed), EMBASE and The Cochrane Library databases, using the medical subject headings (MeSH) terms "systemic lupus erythematosus" AND "high-risk pregnancy" OR "prenatal care." Studies published in English between 2007 and 2017 were included; experimental studies and case reports were excluded. Read More

Cureus 2018 Feb 21;10(2):e2212. Epub 2018 Feb 21.

Sir Seewoosagur Ramgoolam National Hospital.

Cutaneous neonatal lupus erythematosus (NLE) is a rare condition caused by the passive transfer of autoantibodies from mother to fetus. The disease most commonly presents itself with multiple erythematosus annular lesions or arcuate macules. In 10% of the cases, a cardiac anomaly can coexist. Read More

Joint Bone Spine 2018 12 6;85(6):663-668. Epub 2018 Apr 6.

Division of Internal Medicine and Infectious Diseases, Haut-Lévêque Hospital, 33604 Pessac, France. Electronic address:

Objectives: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that primarily affects women of childbearing age. While the impact of hydroxychloroquine (HCQ) on SLE activity and neonatal lupus occurrence has been evaluated in several studies, its role on prematurity and intrauterine growth restriction (IUGR) remains uncertain. The aim of this study was to assess the impact of HCQ exposure on prematurity and IUGR during pregnancy in women with SLE. Read More

J Clin Lab Anal 2018 Jul 26;32(6):e22436. Epub 2018 Mar 26.

Department of Clinical Laboratory, Key Laboratory of Laboratory Medicine of Henan Province, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.

Background: The incidence of dilated cardiomyopathy (DCM) has increased in recent years, and many studies have sought to further improve the general understanding of this condition. Previous studies have demonstrated that some single nucleotide polymorphisms (SNPs) associated with systemic lupus erythematosus also affect susceptibility to DCM, suggesting that immune-related diseases may share similar genetic susceptibility. Recent large-scale and genome-wide association studies have identified NCR3, NOTCH4, CYP1A2, ITGA1, OPRM1, ST8SIA2, and LINC00704 as genetic risk factors associated with cardiac manifestations of neonatal lupus. Read More