新生儿红斑狼疮(NLE)是一种罕见的疾病,由母亲被动传播的自身抗体引起。NLE是一种以皮肤为特征的多器官系统疾病,心脏,血液学,肝胆,和神经表现。本研究旨在回顾NLE婴幼儿及其母亲的各种症状和临床表现。我们对2009年1月至2020年12月在釜山国立大学儿童医院接受检查和治疗的NLE患者及其母亲的病历进行了回顾性审查。27例NLE患者包括13例男性患者(48.1%)和14例女性患者(51.9%)。最常见的症状是皮疹(40.7%),其次是发烧(25.9%),心律失常(14.8%),脾肿大(11.1%),和宫内发育迟缓(7.4%)。7例发热患者有各种器官系统表现,包括皮肤(100%),血液学(71.4%),肝胆(57.1%),中枢神经系统(CNS;28.6%)表现。发热患者中有两名患有无菌性脑膜炎。皮肤,心脏,血液学,肝胆,中枢神经系统受累占44.4%,18.5%,51.9%,40.7%,和22.2%的患者,分别。系统性红斑狼疮(SLE)是最常见的孕产妇疾病(14/27,51.9%)。十名母亲(37.0%)在婴儿被诊断之前没有被诊断出患有任何自身免疫性疾病。其中,三人随后被诊断为SLE,5人被诊断出患有干燥综合征,其中两人仍然没有任何自身免疫性疾病的诊断。发烧是NLE的常见症状;因此,当婴儿没有明确的发烧焦点时,NLE需要考虑,尤其是有皮疹的病例.
Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan National University Children\'s Hospital between January 2009 and December 2020 and their mothers. Twenty-seven patients with NLE comprising 13 male patients (48.1%) and 14 female patients (51.9%) were included. The most common symptom was rash (40.7%), followed by fever (25.9%), arrhythmia (14.8%), splenomegaly (11.1%), and intrauterine growth retardation (7.4%). Seven patients with fever had various organ system manifestations, including cutaneous (100%), hematological (71.4%), hepatobiliary (57.1%), and central nervous system (CNS; 28.6%) manifestations. Two of the febrile patients had aseptic meningitis. Cutaneous, cardiac, hematological, hepatobiliary, and CNS involvement were noted in 44.4%, 18.5%, 51.9%, 40.7%, and 22.2% of the patients, respectively. Systemic lupus erythematosus (SLE) was the most common maternal disease (14/27, 51.9%). Ten mothers (37.0%) had not been diagnosed with any autoimmune disease until their babies were diagnosed. Among them, three were subsequently diagnosed with SLE, five were diagnosed with the Sjögren\'s syndrome, and two of them still had no known diagnosis of any autoimmune disorder. Fever is a common symptom of NLE; thus, when there is no clear focus of fever in infants, NLE needs to be considered, especially in cases with skin rashes.