Neonatal lupus in consecutive pregnancies

Kirsanova T.V., Yarotskaya V.Yu., Ryumina I.I., Timoshina I.V., Potapova A.A., Marycheva N.M.

1) Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow, Russia; 2) Faculty of Fundamental Medicine, M.V. Lomonosov Moscow State University, Moscow, Russia
Background: Neonatal lupus (NL) is usually called a disease of the newborn. The main role in the pathogenesis of NL is attributed to the passive transplacental transfer of maternal anti-Ro/SSA and/or anti-La/SSB autoantibodies. NL is clinically manifested as an isolated skin disease in approximately one half of all the described cases and as an isolated congenital heart block in the other half. Blood, liver, biliary, and other diseases are also described. The most severe presentation of the disease is complete AV block that begins during the second trimester of pregnancy, is irreversible and may require lifelong pacing. Despite the fact that the syndrome is most often detected in children of mothers with Sjögren’s disease, systemic lupus erythematosus (SLE), rheumatoid arthritis, and other rheumatic diseases, the children of asymptomatic mothers can also develop the syndrome at the time of pregnancy.
Case report: The authors describe a clinical case of two consecutive pregnancies in a woman who considers herself healthy and have given birth to their children with NL. Her first child has symptoms that can be attributed to the manifestations of NL, such as thrombocytopenia and splenomegaly; the second child has a cardiac conduction lesion and skin eruption. After her second child’s birth, the mother was examined and found to have anti-Ro/SSA and anti-La/SSB, and anti-DNA antibodies, and a decrease in complement components C3 and C4. Moderate SLE (SLEDAI 2K 8) and Sjögren’s syndrome were diagnosed. At the moment, the mother says that her children are healthy and do not receive treatment; planned ECG and 24-hour ECG Holter monitoring have revealed no abnormalities. Different phenotypes of NL and approaches to its therapy are discussed.
Conclusion: This case is described to enhance the vigilance of physicians of various profiles with regard to the possible occult course of rheumatic diseases, while NL in children becomes an indicator of maternal disease.

Keywords

neonatal lupus
pregnancy
Ro/SSA
La/SSB
thrombocytopenia
splenomegaly
clinical case

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Received 12.01.2021

Accepted 29.06.2021

About the Authors

Tatyana V. Kirsanova, PhD, Senior Researcher of the Department of Transfusiology and Extracorporeal Hemocorrection, V.I. Kulakov NMRC for OG&P,
Ministry of Health of Russia, +7(926)248-45-60, a_tatya@mail.ru, https://orcid.org/0000-0002-6125-590X, 117997, Russia, Moscow, Ac. Oparin str., 4.
Varvara Yu. Yarotskaya, Lomonosov Moscow State University, Faculty of Fundamental Medicine, +7(985)842-59-02, varvara.yarotskaya@gmail.com,
https://orcid.org/0000-0002-9524-0757, 119991, Russia, Moscow, Lomonosovsky Prospekt, 27-1.
Irina I. Ryumina, Dr. Med. Sci., Head of the Department of Pathology of Newborns and Preterm Infants, V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia,
+7(903)770-80-48, i_ryumina@oparina4.ru, i.ryumina@mail.ru, https://orcid.org/0000-0003-1831-887X, 117997, Russia, Moscow, Ac. Oparin str., 4.
Irina V. Timoshina, Ph.D., Researcher of High Risk Pregnancy Department, V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(916)513-48-24,
timoshinairina@yandex.ru, https://orcid.org/0000-0001-6145-4215, 117997, Russia, Moscow, Ac. Oparin str., 4.
Alyona A. Potapova, graduate student of High Risk Pregnancy Department, V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(985)842-59-02,
doc.PotapovaAA@yandex.ru, https://orcid.org/0000-0002-4940-3201, 117997, Russia, Moscow, Ac. Oparin str., 4.
Natalia M. Marycheva, PhD, Dermatologist of Therapeutic Department, V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(985)142-44-72,
n_marycheva@oparina4.ru, 117997, Russia, Moscow, Ac. Oparin str., 4.
Corresponding author: Varvara Yu. Yarotskaya, varvara.yarotskaya@gmail.com

Authors’ contributions: Kirsanova T.V., Yarotskaya V.Yu. – concept and design of the investigation; statistical data processing; writing the text; Yarotskaya V.Yu., Marycheva N.M., Timoshina I.V., Potapova A.A. – material collection and processing; Ryumina I.I. – editing.
Conflicts of interest: The authors declare that there are no conflicts of interest.
Funding: The investigation has not been sponsored.
Patient Consent for Publication: The patient provided informed consent for the publication of her data and associated images.
Authors' Data Sharing Statement: The data supporting the findings of this study are available on request from the corresponding author after approval from the principal investigator.

For citation: Kirsanova T.V., Yarotskaya V.Yu., Ryumina I.I., Timoshina I.V., Potapova A.A., Marycheva N.M. Neonatal lupus in consecutive pregnancies.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2021; 9: 227-231 (in Russian)
https://dx.doi.org/10.18565/aig.2021.9.227-231

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