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Neonatal lupus erythematosus with congenital complete heart block in an asymptomatic mother

1 Department of Pediatrics, Sawai Man Singh Medical College & Hospital, Jaipur, Rajasthan, India
2 Department of Clinical Immunology and Rheumatology, Sawai Man Singh Medical College & Hospital, Jaipur, Rajasthan, India

Date of Submission05-May-2022
Date of Acceptance04-Sep-2022
Date of Web Publication07-Oct-2022

Correspondence Address:
Rohan Grotra,
C-195, Madhuban Colony, I.P. Extension, New Delhi - 110 092
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_92_22

How to cite this URL:
Grotra R, Pansari V, Jain A, Gupta A. Neonatal lupus erythematosus with congenital complete heart block in an asymptomatic mother. Indian J Rheumatol [Epub ahead of print] [cited 2023 Feb 1]. Available from:

Dear Editor,

Neonatal lupus erythematosus (NLE) is a syndrome characterized by skin, cardiac, and systemic abnormalities seen in newborn infants whose mothers have autoantibodies against Ro/SSA and/or La/SSB.[1] Approximately 98% of affected infants have a maternal transfer of autoantibodies against Ro/SSA and La/SSB. Congenital heart block (CHB) is the most serious sequelae of NLE characterized by different degrees of atrioventricular heart block. These autoantibodies damage the myocardium, leading to electrophysiological abnormalities which lead to congestive heart failure.[2] In this report, we present the case of a newborn with isolated CHB born to an asymptomatic mother. A second-born full-term female baby with a birth weight of 2 kg, was referred on day 2 of life, in view of bradycardia since birth. Routine ultrasonography revealed fetal bradycardia in the second trimester but the mother did not follow it up. The mother had no history of rash, ulcers, joint pain, loss of appetite, or any other signs/symptoms suggestive of connective tissue disease. The firstborn baby was normal. On arrival, the baby was pink, active, and breastfeeding well. Transient cutaneous manifestations of NLE such as rash, periorbital erythema, bullous lesions, alopecia, dyspigmentation, and scarring were absent in the baby. On auscultation heart rate of 50–52 beats/min (bpm) and blood pressure of 66/42 mmHg were observed with no signs of heart failure. An electrocardiogram (ECG) showed features of complete third-degree heart block [Figure 1]. Two-dimensional (2D) echocardiography (ECHO) showed no structural abnormality. Mother was euthyroid and was tested for antinuclear antibody and anti-double stranded DNA antibody by ELISA method, both of which were positive. An immunoblot panel done on neonates was positive for anti-Ro (52 kDa) antibody, anti-SSA native (60 kDa) antibody, and anti-SSB antibody. Throughout the hospital stay, the baby was asymptomatic without any hemodynamic instability. Epicardial permanent pacemaker insertion was done on the 11th day of life. Postoperatively, ECG [Figure 2] showed a heart rate of 140 bpm without any heart block. NLE is a rare transplacental passively acquired disorder due to autoantibodies to Ro/SSA and La/SSB which cause damage to the conduction system of the heart.[3] These antibodies may be found in mothers who have been diagnosed with systemic lupus erythematosus (SLE), primary Sjogren's syndrome, antiphospholipid syndrome, or other autoimmune disorders but only cause clinical disease in a minority of babies born to mothers with these autoantibodies. Early detection of CHB and timely management hold the key to the success of the infant's survival. The primary goal of the pacemaker is to prevent sudden cardiac death and to provide symptomatic relief with improvement in functional capacity. Systemic steroids and immunosuppressives are generally not required for NLE.[4]
Figure 1: ECG lead II showing a heart rate of 50 bpm with 3:1 heart block. ECG: Electrocardiogram

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Figure 2: Postoperative ECG after pacemaker placement showing a heart rate of 140 bpm. ECG: Electrocardiogram

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Mothers of infants with NLE, particularly infants with CHB, have a 2–3 fold increased risk of having an affected infant in a subsequent pregnancy. Therefore, carefully monitoring of subsequent pregnancies with serial ultrasonography and ECHO is essential. Treatment with fluorinated glucocorticoids as first-line therapy for first-degree heart block is debatable as some series have shown no impact of therapy or progression despite therapy.[5],[6] Intravenous immunoglobulin and fluorinated glucocorticoids (dexamethasone 4–8 mg/day or betamethasone 3 mg/day) are used for second-degree heart block but are not suggested for third-degree block unless there are other indications.[7] The use of hydroxychloroquine for patients with SLE has been associated with a lower rate of NLE during pregnancy.[8] The current best evidence for the management of CHB in NLE is by fetal 2D ECHO, pacemaker insertion for baby, and management of mothers with a rheumatologist.

Research ethics

Ethical committee approval was not required.

Patient consent

The authors declare that written informed consent was taken for using the reports and images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Research data

The authors hereby declare that the research data may be used in a suitable public repository for accessibility.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Buyon JP, Rupel A, Clancy RM. Neonatal lupus syndromes. Lupus 2004;13:705-12.  Back to cited text no. 1
Eftekhari P, Sallé L, Lezoualc'h F, Mialet J, Gastineau M, Briand JP, et al. Anti-SSA/Ro52 autoantibodies blocking the cardiac 5-HT4 serotoninergic receptor could explain neonatal lupus congenital heart block. Eur J Immunol 2000;30:2782-90.  Back to cited text no. 2
Wisuthsarewong W, Soongswang J, Chantorn R. Neonatal lupus erythematosus: Clinical character, investigation, and outcome. Pediatr Dermatol 2011;28:115-21.  Back to cited text no. 3
Lee LA. Cutaneous lupus in infancy and childhood. Lupus 2010;19:1112-7.  Back to cited text no. 4
Rein AJ, Mevorach D, Perles Z, Gavri S, Nadjari M, Nir A, et al. Early diagnosis and treatment of atrioventricular block in the fetus exposed to maternal anti-SSA/Ro-SSB/La antibodies: A prospective, observational, fetal kinetocardiogram-based study. Circulation 2009;119:1867-72.  Back to cited text no. 5
Michael A, Radwan AA, Ali AK, Abd-Elkariem AY, Shazly SA, Middle-East Obstetrics and Gynecology Graduate Education (MOGGE) Foundation Research Group. Use of antenatal fluorinated corticosteroids in management of congenital heart block: Systematic review and meta-analysis. Eur J Obstet Gynecol Reprod Biol X 2019;4:100072.  Back to cited text no. 6
Jaeggi ET, Silverman ED, Laskin C, Kingdom J, Golding F, Weber R. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol 2011;57:1487-92.  Back to cited text no. 7
Izmirly PM, Kim MY, Llanos C, Le PU, Guerra MM, Askanase AD, et al. Evaluation of the risk of anti-SSA/Ro-SSB/La antibody-associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine. Ann Rheum Dis 2010;69:1827-30.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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