Neonatal lupus: diagnostic difficulties, treatment and prognosis in an extremely premature baby

Abstract

Background. Neonatal lupus (NL) is a rare symptom complex of neonates that develops as a result of tissues' and organs' damage associated with transplacental passage of maternal anti-Ro and anti-La antibodies. The management of infants with heart injury as a result of NL represents great difficulties due to the rarity, heterogeneity of the manifestations of the disease and the lack of treatment protocols.

The aim was to describe a clinical course of NL with severe cardiac manifestations in extremely preterm newborn [gestational age (GA) 25 weeks]; with a relatively favorable outcome at the age of 18 months of life.

Case report. An extremely preterm girl weighing 980 g was born at 25 weeks GA by caesarean section due to persistent fetal bradycardia during routine obstetric ultrasound examination, which was regarded as a sign of fetal distress. The mother had no symptoms of rheumatic diseases. From birth - the baby had bradycardia without a tendency to recovery (heart rate 65-70 beats/min). According to electrocardiography data, grade III atrioventricular block was detected. The diagnosis of NL was confirmed by the presence of anti-Ro antibodies in the neonate and mother. Due to the presence of systolic myocardial dysfunction in the early neonatal period, an systemic steroid therapy was started, which was canceled after persistent hyperglycemia. On the day of life (DOL) 30, an increase of myocardial damage biomarkers was revealed (NT-proBNP, CPK) in the absence of echographic signs of cardiomyopathy, which was regarded as a possible antibody-associated myocardial damage, treatment with glucocorticosteroids was started, the course was interrupted at DOL 42 due to fungal infection. The levels myocardial damage biomarkers decreased to the norm at the age of 56 DOL.

The initial sepsis-checkup confirmed the realization of clinical early onset neonatal sepsis. During the treatment in the NICU, the neonate was diagnosed with disseminated candidiasis and acquired CMV infection with sepsis-like manifestation. Conducted 5 courses of antibacterial and 1 course of antimycotic therapy, taking into account the sensitivity of the detected microflora, antiviral therapy with a positive effect.

The clinical condition of the baby was complicated by the development of DIC syndrome (pulmonary hemorrhage and intraventricular hemorrhage of grade 2), the formation of a moderate form of bronchopulmonary dysplasia, and prolonged functioning of the hemodynamically significant ductus arteriosus.

The duration of respiratory therapy was 75 days (of which invasive mechanical ventilation was 45). The baby survived without major neonatal morbidities. Follow-up at the age of 18 months showed no disabilities nor psychomotor delay; no signs of cardiomyopathy; cardiac function was compensated without indications for cardiac pacing.

Conclusion. Multidisciplinary management, timely detection and reasonable treatment of complications made it possible not only to avoid lethal outcome in an extremely premature baby with comorbidity, including cardiac manifestations of NL, but also to ensure survival without disabling pathology. Probably "aggressive" steroid treatment led to discontinuation of antibody-dependent extranodal myocardial damage and prevention of irreversible postnatal complications of NL. However, this issue remains controversial due to the complications of steroid therapy and should be carefully weighed in terms of the risk/benefit ratio.

Keywords:premature neonate, neonatal lupus, congenital heart block, AV-block, myocardial damage, extremely low birth weight

Funding. The study had no sponsor support.

Conflict of interest. The authors declare no conflict of interest.

Contribution. Concept - Nikitina I.V.; text writing - Nikitina I.V., Krog-Jensen O.A., Nikonets A.D.; illustrations - Krog-Jensen O.A., Nikonets A.D., Belova E.I.; editing - Nikitina I.V., Lenyushkina A.A.

For citation: Nikitina I.V., Krogh-Jensen O.A., Nikonets A.D., Belova E.E., Lenyushkina A.A. Neonatal lupus: diagnostic difficulties, treatment and prognosis in an extremely premature baby. Neonatologiya: novosti, mneniya, obuchenie [Neonatology: News, Opinions, Training]. 2021; 9 (2): 40-53. DOI: https://doi.org/10.33029/2308-2402-2021-9-2-40-53 (in Russian)

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CHIEF EDITOR
CHIEF EDITOR
Degtyarev Dmitriy Nikolaevich
Doctor of Medical Sciences, Professor, Deputy Director for Scientific Research of the V.I. Kulakov Obstetrics, Gynecology and Perinatology National Medical Research Center of Ministry of Healthсаre of the Russian Federation, Head of the Chair of Neonatology at the Clinical Institute of Children's Health named after N.F. Filatov, I.M. Sechenov First Moscow State Medical University, Chairman of the Ethics Committee of the Russian Society of Neonatologists, Moscow, Russian Federation

ORCID iD 0000-0001-8975-2425

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