ABSTRACT
Objective To summarize the clinical features of neonatal lupus erythematosus (NLE) and to have a better clinical understanding. Methods We retrospectively analyzed the clinical data of 13 patients with NLE who were hospitalized in Fujian Provincial Maternity and Children's Hospital from September 2010 to September 2017. The pathogenesis, clinical manifestations, laboratory examinition, management and long term outcomes of these babies were summarized, and the relevant literatures were also reviewed. Descriptive statistical analysis was performed. ResuLts The 13 NLE cases included eight boys and five girls. Among them, skin lesions, cardiac impairment, hematological problems, hepatobiliary system damage, central nervous system involvement and renal function damage occurred in eight, seven (six cases were atrioventricular block), seven, three, one and one case, respectively. Antinuclear antibodies and anti-Sjogren's syndrome antigen A antibodies were positive in all neonates, anti-Sjogren's syndrome antigen B antibodies were positive in 11, and anti-double-stranded DNA antibodies were positive in two cases. Among the 13 mothers, three were diagnosed with Sjogren's syndrome and two had systemic lupus erythematosus(SLE) before pregnancy, two were diagnosed with Sjogren's syndrome and one developed SLE during pregnancy. Eight babies with skin lesions were asked to avoid light and the skin rash all gradually receded within 1-6 months after birth. Five cases with thrombocytopenia were treated with intravenous immunoglobulin and one anemic baby received erythrocyte transfusion. Within 2 to 3 months, the impaired blood system in these babies were back to normal. For the three babies with abnormal liver functions, hepatic protectants and jaundice relieving agents were given, and 2 to 6 months later they recovered. The 13 patients were followed up for five months to seven years, among which, seven improved with normal growth and development; five still had grade Ⅲ atrioventricular block; one installed an atrial pacemaker at 11 months. Three mothers who were asymptomatic during pregnancy were found to have autoimmune diseases after their babies were diagnosed with NLE, including one case of Sjogren's syndrome and two of SLE. ConcLusions NLE is mainly characterized by skin lesions and congenital heart block, while liver, blood system, central nervous system and other organs may also be involved. For high-risk mothers and babies, timely autoantibody screening and relevant examinations are suggested for early diagnosis and interventions. In addition, long term follow-up is required for affected cases.