Abstract
Neonatal lupus erythematosus is a clinical syndrome that occurs in the babies of mothers who are positive for anti-Ro and anti-La autoantibodies and characterized by cardiac and cutaneous manifestations. The risk of developing neonatal lupus during first pregnancy is about 2% but the risk of recurrence in mothers with past history of neonatal lupus is higher, up to 18–20%. Cardiac involvement in the form of complete heart block is the most dreaded and is usually irreversible and is associated with increased morbidity and mortality. The cardiac manifestations can develop as early as 16 weeks of gestation; hence, in utero cardiac screening is recommended for at-risk fetuses starting from 16 weeks. Hydroxychloroquine has been shown to prevent the development of cardiac manifestations but the role of other treatments like fluorinated steroids, intravenous immunoglobulin, and plasma exchange is still doubtful. Pediatric cardiologist should be involved in the management as infants with complete heart block will need pacemaker implantation immediately after birth.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Franceschini F, Cavazzana I (2005) Anti-Ro/SSA and La/SSB antibodies. Autoimmunity 38:55–63
Palmeira P, Quinello C, Silveira-Lessa AL et al (2012) IgG placental transfer in healthy and pathological pregnancies. Clin Dev Immunol 2012:985646
Izmirly P, Saxena A, Buyon JP (2017) Progress in the pathogenesis and treatment of cardiac manifestations of neonatal lupus. Curr Opin Rheumatol 29:467–472
Hon KL, Leung AKC (2012) Neonatal lupus erythematosus. Autoimmune Dis 2012:301274
Gordon P, Khamashta MA, Rosenthal E et al (2004) Anti-52 kDa Ro, anti-60 kDa Ro, and anti-La antibody profiles in neonatal lupus. J Rheumatol 31:2480–2487
Brito-Zeron P, Izmirly PM, Ramos-Casals M et al (2015) The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 11:301–312
Brucato A, Cimaz R, Caporali R et al (2011) Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clin Rev Allergy Immunol 40:27–41
Martínez-Sánchez N, Perez-Pinto S, Robles-Marhuenda A et al (2017) Obstetric and perinatal outcome in anti-Ro/SSA-positive pregnant women: a prospective cohort study. Immunol Res 65:487–494
Costedoat-Chalumeau N, Amoura Z, Lupoglazoff JM et al (2004) Outcome of pregnancies in patients with anti-SSA/Ro antibodies a study of 165 pregnancies, with special focus on electrocardiographic variations in the children and comparison with a control group. Arthritis Rheum 50:3187–3194
Jaeggi E, Laskin C, Hamilton R et al (2010) The importance of the level of maternal anti-Ro/SSA antibodies as a prognostic marker of the development of cardiac neonatal lupus erythematosus: a prospective study of 186 antibody-exposed fetuses and infants. J Am Coll Cardiol 55:2778–2784
Lehman TJA, Reichlin M, Santner TJ et al (1989) Maternal antibodies to Ro (SS-A) are associated with both early onset of disease and male sex among children with systemic lupus erythematosus. Arthritis Rheum 32:1414–1420
Martin V, Lee LA, Askanase AD et al (2002) Long-term follow-up of children with neonatal lupus and their unaffected siblings. Arthritis Rheum 46:2377–2383
Rivera TL, Izmirly PM, Birnbaum BK et al (2009) Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Ann Rheum Dis 68:828–835
Saxena A, Izmirly PM, Mendez B et al (2014) Prevention and treatment in utero of autoimmune associated congenital heart block. Cardiol Rev 22:263–267
Friedman DM, Llanos C, Izmirly PM et al (2010) Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: results of a multicenter, prospective, open-label clinical trial. Arthritis Rheum 62:1138–1146
Pisoni CN, Brucato A, Ruffatti A et al (2010) Failure of intravenous immunoglobulin to prevent congenital heart block: findings of a multicenter, prospective, observational study. Arthritis Rheum 62:1147–1152
Izmirly PM, Kim MY, Llanos C et al (2010) 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 69:1827–1830
Izmirly PM, Costedoat-Chalumeau N, Pisoni CN et al (2012) Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation 126:76–82
Levy RA, Vilela VS, Cataldo MJ et al (2001) Hydroxychloroquine in lupus pregnancy: a double-blind placebo-controlled study. Lupus 10:401–404
Epstein AE, DiMarco JP, Ellenbogen KA et al (2013) 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines and the Heart Rhythm Society. J Am Coll Cardiol 61:e6–e75
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Singapore Pte Ltd.
About this chapter
Cite this chapter
Naidu, G.S.R.S.N.K., Adarsh, M.B. (2020). Management and Monitoring of Anti-Ro/La positive Mother. In: Sharma, S. (eds) Women's Health in Autoimmune Diseases. Springer, Singapore. https://doi.org/10.1007/978-981-15-0114-2_17
Download citation
DOI: https://doi.org/10.1007/978-981-15-0114-2_17
Published:
Publisher Name: Springer, Singapore
Print ISBN: 978-981-15-0113-5
Online ISBN: 978-981-15-0114-2
eBook Packages: MedicineMedicine (R0)