目的:新生儿系统性红斑狼疮(NLE)是一种获得性自身免疫性疾病。渗出的存在,如胸腔积液和心包积液,是罕见的。本研究有助于探讨NLE合并积液患儿的临床特征和进展。
方法:对2011年1月1日至2023年12月31日在苏州大学附属儿童医院和苏州市立医院确诊的NLE患者的临床资料进行回顾性分析。根据是否存在积液,将NLE患者分为积液组和非积液组。此外,对两组新生儿的临床资料进行比较和调查。
结果:11例(11/45,24.44%)NLE患者出现积液,如胸腔积液,睾丸鞘膜积液,腹腔积液,心包积液,和脑积水.积液患者的其他器官为皮肤,胃肠,血液学,心脏,和神经学。在有积液的患者中,5例SLE孕妇,干燥综合征2例,一例光过敏症状,3名孕妇均无产前自身免疫性疾病史。孕母自身免疫性疾病在怀孕前缓解,或稳定的低疾病活动。7例患者抗SSA阳性,其中5种抗SSA和抗SSB双阳性。与非积液组相比,积液组患者的乳酸脱氢酶水平明显升高,肌酸激酶,和纤维蛋白原,显著降低血小板,总蛋白质,和白蛋白。这些患者更有可能患有血小板减少症和凝血异常。Logistics回归分析表明,有积液的NLE患者血清总蛋白水平降低的可能性更大。所有有积液的NLE患者都有积液的自我吸收。
结论:在我们的研究中,24.44%的患者有积液。有积液的NLE患者更可能有血液学受累和更多的炎症反应。NLE患者的积液通常是自我吸收,严重病例可用非甾体抗炎药/类固醇治疗.要点•NLE患者合并有积液和自限性,以胸腔积液最为常见.•合并积液的NLE患者有更多的炎症反应,血常规及生化相关指标显著异常。
OBJECTIVE: Neonatal systemic lupus erythematosus (NLE) is an acquired autoimmune disease. The presence of effusions, such as pleural
effusion and pericardial
effusion, is rare. The present study helped investigate the clinical characteristics and progression of children with NLE combined with effusions.
METHODS: Clinical data of patients diagnosed with NLE were retrospectively collected and analyzed from January 1, 2011, to December 31, 2023, at the Children\'s Hospital of Soochow University and Suzhou Municipal Hospital. Patients with NLE were divided into
effusion and non-
effusion groups based on the presence of
effusion. Moreover, the clinical data of the newborns in both groups were compared and investigated.
RESULTS: Eleven (11/45, 24.44%) NLE patients had effusions, such as pleural effusion, testicular hydrocele, peritoneal
effusion, pericardial
effusion, and hydrocephalus. Other organs involved in effusion patients were cutaneous, gastrointestinal, hematologic, cardiac, and neurological. Among the patients with effusion, five cases of SLE in pregnant mothers, two cases of Sjogren\'s syndrome, one case of photoallergic symptoms, and three of pregnant mothers with no history of antenatal autoimmune disease. Pregnant mother\' autoimmune disease in remission prior to pregnancy, or stable low disease activity. Seven patients were positive for Anti-SSA, five of which were double positive for Anti-SSA and Anti-SSB. Compared with the non-
effusion group, the
effusion group patients had significantly higher lactate dehydrogenase, creatine kinase, and fibrinogen, significantly lower platelets, total protein, and albumin. These patients were likelier to have thrombocytopenia and coagulation abnormalities. Logistics regression analysis demonstrated that NLE patients with effusions are more likely to have decreased serum total protein levels. All NLE patients with
effusion have self-resorption of the
effusion.
CONCLUSIONS: 24.44% of patients had effusions in our study. NLE patients with effusion are more likely to have hematologic involvement and a more inflammatory response. The effusion in NLE patients is usually self-resorption, severe cases can be treated with nonsteroidal anti-inflammatory drugs/steroids. Key Points • NLE patients combined with effusions and were self-limiting, with pleural effusion being the most common. • NLE patients combined with effusions have a more inflammatory response, significant abnormalities in the blood routine and biochemical-related indexes.