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<p>Neonatal lupus erythematosus (NLE) is a&#xa0;rare congenital autoimmune disease of the fetus and newborn mediated by transplacental maternal autoantibodies, particularly anti-SSA/Ro and anti-SSB/La, which are primarily found in patients with systemic lupus erythematosus and Sjögren’s syndrome. Clinical manifestations are heterogeneous and may affect the skin; liver; blood; heart; and, rarely, the central nervous system. While most manifestations are self-limiting, congenital third-degree atrioventricular block (AV) is irreversible and associated with high morbidity and mortality. Pathogenetically, antibody-mediated inflammatory reactions in fetal tissues—modulated by genetic and environmental factors—play a&#xa0;central role. Treatment depends on the specific manifestation: cutaneous, hepatobiliary, hematological, and neurological changes are treated predominantly symptomatically, whereas third-degree AV block usually requires pacemaker implantation. Early prenatal diagnosis and interdisciplinary management are essential. Early treatment with hydroxychloroquine during pregnancy can significantly reduce the risk of cardiac manifestations.</p>

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Neonataler Lupus erythematodes – eine interdisziplinäre Herausforderung

  • 期刊论文

  • 发表日期：

    2026年7月28日

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  • Angelika Hammer

摘要

Neonatal lupus erythematosus (NLE) is a rare congenital autoimmune disease of the fetus and newborn mediated by transplacental maternal autoantibodies, particularly anti-SSA/Ro and anti-SSB/La, which are primarily found in patients with systemic lupus erythematosus and Sjögren’s syndrome. Clinical manifestations are heterogeneous and may affect the skin; liver; blood; heart; and, rarely, the central nervous system. While most manifestations are self-limiting, congenital third-degree atrioventricular block (AV) is irreversible and associated with high morbidity and mortality. Pathogenetically, antibody-mediated inflammatory reactions in fetal tissues—modulated by genetic and environmental factors—play a central role. Treatment depends on the specific manifestation: cutaneous, hepatobiliary, hematological, and neurological changes are treated predominantly symptomatically, whereas third-degree AV block usually requires pacemaker implantation. Early prenatal diagnosis and interdisciplinary management are essential. Early treatment with hydroxychloroquine during pregnancy can significantly reduce the risk of cardiac manifestations.