Prenatal predisposing factors associated with neonatal lupus erythematosus

Objectives To identify the prenatal predisposing factors related to neonatal lupus erythematosus (NLE). Materials and Methods A retrospective case-control study was made of 131 pregnant women with positive anti-Ro or anti-La autoantibodies and known neonatal outcomes between January 2002 and December 2019 at Siriraj Hospital, Bangkok, Thailand. There were 101 unaffected neonates and 30 NLE cases confirmed postnatally. Demographic and clinical data of the mothers and neonates with and without NLE were statistically compared. Results NLE was diagnosed in 30 out of 131 cases. A multivariate analysis identified the following significant factors for NLE: maternal anti-La antibodies (odds ratio (OR), 3.591; p = 0.030); and maternal treatment with either hydroxychloroquine (OR, 0.082; p = 0.001) or prednisolone (OR, 0.136; p = 0.017). Of the significant variables examined in the multivariate analysis models, high levels of maternal anti-La antibodies were found to be the strongest predictor of noncardiac NLE (OR, 4.558; p = 0.032), while a female baby was significantly higher in pregnancies complicated by cardiac NLE (OR, 5.374; p = 0.046). Hydroxychloroquine still provided a protective effect for both cardiac and noncardiac NLE ( p = 0.039 and 0.032, respectively). Conclusions The maternal anti-La antibodies were a beneficial predictor for NLE, especially as their high titers were influentially associated with noncardiac features. A female fetus seemed to present an increased risk for developing a congenital heart block. Nevertheless, the treatment with hydroxychloroquine during the pregnancies demonstrated a potentially protective factor against both cardiac and noncardiac manifestations.

Coexistence of Neonatal Lupus Erythematous and Sturge–Weber Syndrome

Neonatal lupus erythematous (NLE) is a rare condition presented by lupus dermatitis shortly after birth or later following sun exposure. Sturge–Weber syndrome (SWS) is also an uncommon congenital condition characterized by extensive capillary malformation and ophthalmic and/or neurologic involvement. Here, we describe the first case of coexistence of NLE and SWS which posed a significant diagnostic challenge to clinicians.

Complete heart block in neonatal lupus: a forgotten cause of fetal bradycardia

The most common cause of congenital heart block (CHB) is neonatal lupus, an acquired autoimmune disease caused by transplacental transfer of maternal antibodies to the fetus. A full-term female neonate was admitted to neonatal intensive care unit for severe bradycardia with stable haemodynamics. The mother, showing no clinical symptoms or any particular history, was transferred to our tertiary centre for profound fetal bradycardia. At birth, the infant’s ECG showed a third-degree atrioventricular block and echocardiography was normal. Cardiac neonatal lupus was confirmed with positive maternal anti-Ro antibodies. Under close monitoring, the infant tolerated the bradycardia well (median 67 beats per minute (bpm)) and was discharged on day 6 of life. There was no indication for pacemaker, but she would be on regular follow-up with a paediatric cardiologist. This article holds an important insight as it is the first confirmed case of autoimmune CHB in Cambodia in which the mother’s antibody was found only after diagnosis on the neonate.

Neonatal lupus: a clinical challenge

Neonatal lupus is an uncommon entity. The main manifestations are cutaneous and cardiac. It is caused by transplacental passage of maternal antibodies (anti-Ro/SSA or anti-La/SSB), and the diagnosis is made by its detection in the mother or child. The authors present a case of a 4-month-old female infant, with a cutaneous eruption since she was 2 months old. She had no relevant personal or family history. Analytically she had an increase in liver enzymes. The histological aspect of the skin biopsy led to an autoimmunity study on the mother and infant, both of which had positive anti-Ro/SSA antibodies, confirming the diagnosis of neonatal lupus. Cardiological study was normal. The skin lesions resolved during the first year of life. Skin lesions are the most frequent non-cardiac clinical manifestation of neonatal lupus, and they are self-limited. When there is no family history, nor cardiac involvement, the diagnosis can be challenging.

P094 “Neonatal lupus”: a unique name for very different realities

Background Neonatal lupus is a rare condition linked to the maternal-fetal transmission of maternal anti-SSA and/or anti-SSB antibodies, more rarely anti-U1-RNP. The most frequent clinical manifestations are cardiac and cutaneous, more rarely hematological (thrombocytopenia, leuco-neutropenia), hepatic (cholestasis), neurological (spastic paresis, lymphocytic meningitis) or renal. We report two observations of neonatal lupus: a classic form, and a rare form. Observations First case: A female newborn, premature of 37 weeks, from a first-degree consanguineous marriage, having a mother followed for systemic lupus erythematosus, with poor therapeutic compliance, presented at H1 of life with neonatal respiratory distress. The clinical examination revealed severe bradycardia at 67 Bpm. The electrocardiogram showed complete atrioventricular block, with a moderate pericarditis on the echocardiography, minimal tricuspid insufficiency, interatrial communication, and a 6 mm foramen oval with LR shunt. The immunological test had objectified positive antinuclear, anti-SSA and anti-SSB antibodies. An implementation of a pacemaker with inter-atrial communication ligation were performed successfully. The evolution was marked by the appearance of a malar erythema with generalized lesions of discoid lupus at the age of 14 months, treated with local corticosteroid therapy, with good outcomes. The control immunological workup was negative at 18 months. Second case: A 2-month-old boy from a non-consanguineous marriage with a mother followed for Sjögren's syndrome was admitted for a symptomatology dating back to birth marked by the appearance of diffuse petechial purpura. Clinically, the infant presented diffuse discoid macules throughout the body. The somatic examination, particularly cardiovascular, was normal. The biological workup showed haemolytic anaemia with a positive coombs test and severe thrombocytopenia. The electrocardiogram and echocardiography were normal. The immunological workup had objectified positive anti-SSA, anti-SSB and anti-nuclear antibodies. The infant was treated by oral corticosteroids (prednisolone) for 4 months, with good outcomes. The control immunological workup was negative at 6 months. Conclusion The clinical manifestations of neonatal lupus are polymorphic, and the only one that may expose to life threat is complete atrioventricular block. All mothers with known lupus or with positive anti-SSA autoantibodies should be monitored during pregnancy with repeated ultrasounds.

Pulmonary Involvement in Neonatal Lupus from Clinical Diagnoses to Autopsy Findings: A Case Report

Background: Neonatal lupus erythematosus (NLE) is an uncommon immunemediated disease caused by the transplacental passage of maternal autoantibodies. In this paper, we described a case of a newborn with NLE involving lung damage from clinical diagnoses to autopsy findings. Case presentation: The mother had no history of immune system disease; however, the test for antinuclear antibodies was positive. A boy was delivered spontaneously at 36+2 weeks’ gestation without asphyxia (birth weight: 1.21 kg, length: 38 cm, head circumference: 26 cm). Soon after birth, intermittent shortness of breath occurred, followed by cyanosis and severe hypoxemia. Blood tests suggested that the infant was positive for anti-nuclear antibodies. Although multiple respiratory support methods were used, death could not be avoided and occurred at 6.5 days due to cardiopulmonary failure. The results of the autopsy revealed diffuse interstitial inflammation and interstitial fibrosis in both lungs, manifesting as telangiectasia, hyperemia, alveolar cavity dilatation and fusion, and obvious interstitial widening with chronic inflammatory cell infiltration. The newborn was finally diagnosed with nonspecific interstitial pneumonia with fibrosis caused by congenital systemic lupus erythematosus. Conclusions: Pulmonary involvement with NLE is a diagnostic challenge at present. Close attention should be given to newborns whose mothers have systemic lupus erythematosus.