Abstract
AIM: This review aims to summarize the epidemiology, pathogenesis, clinical features, management, prognosis and regression of Neonatal lupus erythematosus (NLE) with a view to providing directions for standardized diagnosis, treatment and further research. METHODS: We conducted a comprehensive literature review of NLE. NLE-related peer-reviewed papers were searched through PubMed/Medline were searched up to November 2024. RESULTS: NLE is a rare acquired autoimmune disease (AD) linked to organ damage from maternal autoantibodies crossing the placenta to the foetus. However, not all mothers have ADs or associated antibodies. The disease involves autoantibody-induced inflammation, apoptosis, fibrosis, calcium channel dysregulation in cardiomyocytes, and increased interferon expression. NLE incidence shows no sex difference, but there is a differential distribution of clinical features across ethnic groups. The frequency of organ involvement in NLE patients is more common in the cutaneous and cardiac. NLE also affects the haematological and hepatobiliary systems, and some patients may experience neurological and endocrinological involvement. Steroids and immunoglobulins can aid in the recovery of some patients. Proper use of antimalarials during prenatal and gestational periods may prevent or improve the prognosis of patients with congenital heart block (CHB). Implantation of a pacemaker is effective in maintaining cardiac function in children with complete atrioventricular block. Symptoms associated with NLE may improve with antibody depletion, but some patients may experience sequelae such as irreversible CHB, neuropsychiatric disorders and developmental delays. Universal screening for autoantibodies to Sjögren syndrome A or B autoantigens should be offered to women of childbearing age experiencing desiccation syndrome. Antibody-positive individuals require appropriate reproductive counselling and advice, along with close foetal monitoring starting at 16 weeks of gestation and postnatal prognostic follow-up. CONCLUSION: Epidemiologic investigations and clinical studies on NLE are currently inadequate, and large-scale epidemiologic investigations, prospective clinical studies, and basic research are needed in the future to improve the understanding of the disease and the standardization of its clinical management.