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British Journal of Healthcare and Medical Research - Vol. 9, No. 1

Publication Date: February, 25, 2022

DOI:10.14738/jbemi.91.11869. Aamri, H., Hazzab, N., Bennaoui, F., Slitine, N. E. I., & Maoulainine, F. M. R. (2022). Neonatal Lupus: 5 Pediatric Cases. British Journal

of Healthcare and Medical Research, 9(1). 166-171.

Services for Science and Education – United Kingdom

Neonatal Lupus: 5 Pediatric Cases

Aamri H.

Neonatal intensive care unit, Mother-Child Hospital

CHU Mohammed VI, Marrakech, Morocco

Childhood, Health and Development Research Unit

Faculty of Medicine, Cadi-Ayyad University, Marrakech, Morocco

Hazzab N.

Neonatal intensive care unit, Mother-Child Hospital

CHU Mohammed VI, Marrakech, Morocco

Childhood, Health and Development Research Unit

Faculty of Medicine, Cadi-Ayyad University, Marrakech, Morocco

Bennaoui F.

Neonatal intensive care unit, Mother-Child Hospital

CHU Mohammed VI, Marrakech, Morocco

Childhood, Health and Development Research Unit

Faculty of Medicine, Cadi-Ayyad University, Marrakech, Morocco

N. El Idrissi Slitine

Neonatal intensive care unit, Mother-Child Hospital

CHU Mohammed VI, Marrakech, Morocco

Childhood, Health and Development Research Unit

Faculty of Medicine, Cadi-Ayyad University, Marrakech, Morocco

Maoulainine F. M. R.

Neonatal intensive care unit, Mother-Child Hospital

CHU Mohammed VI, Marrakech, Morocco

Childhood, Health and Development Research Unit

Faculty of Medicine, Cadi-Ayyad University, Marrakech, Morocco

ABSTRACT

Neonatal lupus erythematosus is a rare condition that manifests itself in different

types of damage, whether cutaneous, hepatic, hematological, neurological or even

cardiac. We report here five cases of neonatal lupus occurring in newborns, two of

them from completely asymptomatic mothers and revealed by cardiac involvement

and one case revealed by skin involvement.

Key words: Neonatal lupus erythematosus; congenital heart block; newborn; antinuclear

antibodies

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167

Aamri, H., Hazzab, N., Bennaoui, F., Slitine, N. E. I., & Maoulainine, F. M. R. (2022). Neonatal Lupus: 5 Pediatric Cases. British Journal of Healthcare

and Medical Research, 9(1). 166-171.

URL: http://dx.doi.org/10.14738/jbemi.91.11869

INTRODUCTION

Neonatal lupus erythematosus is manifested by cutaneous, hematological, hepatic or cardiac

involvement including possible congenital atrioventricular block (BAVc) occurring in a heart

free from malformative heart disease, dilated cardiomyopathy and endocardial fibroelastosis.

This rare syndrome is consistently associated with the presence in the mother of anti-SSA / Ro

and / or anti-SSB / La antibodies. Skin involvement has been rarely reported in association with

the isolated anti-RNP antibody.

Cutaneous and systemic damage is transient, unlike cBAV which is definitive and is associated

with morbidity and mortalitywhichmakethis syndrome soserious.

We report five observations of LN occurring in newborns (NB) including two from completely

asymptomatic mothers and one newborn from a motherfollowed for Gougerot-Sjögren

syndrome.

OBSERVATIONS

Observation 1

A femal newborn, from a 37-year-old mother with no pathological history. Poorly monitored

pregnancy conducted at 34 weeks. Cesarean delivery for extreme fetal bradycardias. Having

presented at birth with neonatal respiratory distress associated with bradycardia at 65b / min,

a complete assessment was carried out, a complete blood count objectifying anemia at 11.1g /

dl, without leukopenia or thrombocytopenia. A correct hepatic and renal workup and positive

anti-nuclear antibodies, positive anti-SSA (Ro52 and Ro60) and positive anti-RNP. A chest x-ray

objectified an aspect in favor of an early neonatal infection with pulmonary localization. An ECG

objectified a 3rd degree AVB. A cardiac ultrasound showed a muscle VIC of 2 mm, an

aneurysmaloval foramen. The evolution was marked by the appearance of cutaneous

involvement at the age of 1 and a half made of a butterfly wing erythema of the face. The mother

has a stable antibody level with no associated clinical manifestations.

Observation 2

A male nexborn, from a 42-year-old mother, with the notion of 1st degree consanguinity, having

a miscarriage as a history. Resulting from a well-followed pregnancy and carried to term and

delivery by cesarean section for suspected heart disease due to fetal bradycardia. The newborn

presented at birth a bradycardia at 50 b / min associated with respiratory distress on the 2/10

side according to the silverman score for which he was hospitalized in neonatal intensive care.

A complete workup was carried out with a normal blood count, a renal and hepatic workup

without abnormality and anti-nuclear antibodies positive to 1/320 of the speckled type, anti- SSA (Ro52 and Ro60) positive. An ECG objectified a 3rd degree AVB. A chest x-ray objectified

cardiomegaly with an ICT of 0.64. A cardiac ultrasound showed a very altered global and

segmental contractility, a foramen ovale versus CIA and a membrane on the aortic arch

suggesting a coarctation of the aorta. The course was marked by the worsening of his

bradycardia and his respiratory distress. The newborn died after a four-day stay in intensive

care. The mother was diagnosed at this age and currently followed with stable immunological

and clinical status.

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British Journal of Healthcare and Medical Research (BJHMR) Vol 9, Issue 1, February - 2022

Services for Science and Education – United Kingdom

Observation 3

A male newborn, from a normal pregnancy carried to term, and a mother followed for suspected

lupus in front of rheumatoid purpura + dry syndrome + positive ANA with anti-SSA and anti- SSB positive. Hospitalized in neonatal intensive care at day 6 of life for a postnatal infection

before: thrombocytopenia and a positive CRP. An infectious assessment was performed

showing a urinary tract infection with Escherichia Coli. Put on antibiotic treatment for 10 days.

The course was marked by the persistence of thrombocytopenia and the appearance of rounded

erythematous papular lesions, covered by fine scales (figure 1). The count showed

thrombocytopenia at 58,000 with anemia of 11.5 normochromium, normocytic without

leukopenia. ECG and ETT did not show any abnormalities. NAAs are 1/640 positive with

positive anti-SSA and anti-SSB. The evolution was towards the regression of symptoms in 4

months. The mother iswatched, the hindsight is one year.

Observation 4

It is the child Nour Mallak, aged 4, asymptomatic mother. She presented for a cardiology

consultation for bradycardia. On examination, the child presented with bradycardia at 59b /

min without other associated signs, in particular no skin involvement. A lupus assessment was

requested which objectified positive anti-nuclear antibodies. The indication of a pacemaker

was asked but for lack of means not achieved. The child is stable and bradycardia is tolerated.

Figure 1: Skin involvement made up of roundederythematouspapularlesions, coveredwith fine

scales

Observation 5

A femal newborn, from a mother followed for lupus. He was hospitalized in neonatal intensive

care for fetal bradycardias + IUGR. An ECG objectified a grade 3 AVB. The AANs were positive

at 1/640 of speckled type, anti-SSA positive (Ro52 + Ro60) at 100IU / ml and anti-SSB positive

at 100IU / ml. The biological assessment, in particular the CBC and the hepatic assessment,

were normal. He presented a nosocomial infection following his hospitalization in intensive

care.