Division of Allergy, Asthma and Immunology, Thomas Jefferson University, Nemours/AI duPont Hospital for Children, Wilmington, DE 19803, USA.
J Autoimmun. 2012 May;38(2-3):J223-38. doi: 10.1016/j.jaut.2011.11.018. Epub 2012 Mar 7.
Neonatal autoimmune diseases are distinctly rare. Most neonatal autoimmune diseases result from the transplacental transfer of maternal antibodies directed against fetal or neonatal antigens in various tissues. In neonatal lupus, the heart seems to be particularly susceptible. Primary autoimmunity in newborns, with the exception of familial autoinflammatory diseases, is virtually non-existent. The pathophysiologic basis for the development of neonatal autoimmunity is not entirely clear, but differences in the neonatal immune system compared with the adult immune system, as well as unique characteristics of target antigens in the newborn period may be important factors. Neonatal lupus is the most common presentation of autoimmunity in the newborn. But the characteristics defining neonatal lupus are not well defined and the presentation of neonatal lupus differs from that of classical lupus. Other neonatal autoimmune diseases involving the interaction between maternal antibodies and fetal/neonatal antigens include neonatal anti-phospholipid syndrome, Behcet's disease, neonatal autoimmune thyroid disease, neonatal polymyositis and dermatomyositis, neonatal scleroderma and neonatal type I diabetes mellitus. While autoantibodies have been detected in patients with neonatal autoimmune disease, the pathogenic role of autoantibodies has not been well defined. Other mechanisms may play a role in the development of neonatal autoimmunity, including fetal/maternal microchimerism and aberrant apoptosis of fetal cells. The autoinflammatory syndromes are a completely different category, but are also included in discussion of neonatal autoimmune diseases. The autoinflammatory syndromes include the cryopyrin associated periodic syndromes (CAPS) - familial cold autoinflammatory syndrome (FCAS), neonatal onset multisystem inflammatory disease (NOMID) and Muckle-Wells syndrome, which all share a common pathophysiologic mechanism.
新生儿自身免疫性疾病极为罕见。大多数新生儿自身免疫性疾病是由于母体抗体通过胎盘转移到胎儿或新生儿各种组织中的胎儿或新生儿抗原引起的。在新生儿狼疮中，心脏似乎特别容易受到影响。除家族性自身炎症性疾病外，新生儿原发性自身免疫实际上是不存在的。新生儿自身免疫发生的病理生理基础尚不完全清楚，但新生儿免疫系统与成人免疫系统的差异，以及新生儿期靶抗原的独特特征可能是重要因素。新生儿狼疮是新生儿自身免疫最常见的表现。但是，定义新生儿狼疮的特征并不明确，并且新生儿狼疮的表现与经典狼疮不同。其他涉及母体抗体与胎儿/新生儿抗原相互作用的新生儿自身免疫性疾病包括新生儿抗磷脂综合征、贝赫切特病、新生儿自身免疫性甲状腺疾病、新生儿多发性肌炎和皮肌炎、新生儿硬皮病和新生儿 1 型糖尿病。虽然在患有新生儿自身免疫性疾病的患者中已经检测到自身抗体，但自身抗体的致病作用尚未得到很好的定义。其他机制可能在新生儿自身免疫的发展中起作用，包括胎儿/母体嵌合体和胎儿细胞的异常凋亡。自身炎症性综合征是一个完全不同的类别，但也包括在新生儿自身免疫性疾病的讨论中。自身炎症性综合征包括 Cryopyrin 相关周期性综合征（CAPS）-家族性冷自身炎症综合征（FCAS）、新生儿发病多系统炎症性疾病（NOMID）和 Muckle-Wells 综合征，它们都具有共同的病理生理机制。
