NEONATAL LUPUS SYNDROME – AN OVERVIEW OF THE PATHOGENESIS, CLINICAL FEATURES, AND MANAGEMENT APPROACH

Authors

  • Nikolina Zdraveska University Children’s Hospital, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia

Keywords:

neonatal lupus erythematosus, congenital heart block, cutaneous lupus syndrome, antibodies, autoimmune disease

Abstract

Neonatal lupus erythematosus (NLE) is a rare immune-mediated condition that affects newborns due to the transplacental transfer of maternal antibodies, specifically anti-Ro/SSA and/or anti-La/SSB. Seropositive mothers may have known or undiagnosed autoimmune diseases, and many of them remain asymptomatic.

NLE is associated with various phenotypic characteristics and can affect multiple organ systems, including the skin, heart, blood, and liver. The most severe manifestation of NLE is a complete atrioventricular block, which may occur in utero and is irreversible. Cutaneous manifestations resemble the rash seen in subacute lupus erythematosus and are generally benign and self-limiting, typically resolving completely within four to six months.

During the neonatal period, these rashes are frequently misdiagnosed and confused with other common erythematous conditions, particularly when the mother is asymptomatic.

Although NLE typically has a benign course, its clinical significance is highlighted by a tenfold increased risk of recurrence in subsequent pregnancies, as well as its association with more severe clinical outcomes. This article provides a comprehensive review of pathogenesis, clinical manifestations and management of NLE aiming to serve as a valuable reference for clinical practitioners in the field.

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Published

2025-06-12

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Review Article