Horng Y C, Chou Y H, Tsou Yau K I
Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1992 Sep-Oct;33(5):372-5.
Since 1981 when anti-Ro (SS-A) and/or anti-La (SS-B) antibodies were described to be present in infants with neonatal lupus erythematosus (NLE) and their mothers, subsequent studies have demonstrated the almost universal association of NLE with either or both of these autoantibodies. To our best knowledge, three cases of NLE were reported to be negative in anti-Ro (SS-A) and anti-La (SS-B) antibodies. We report one infant born to a mother with systemic lupus erythematosus (SLE). He had neonatal pancytopenia (thrombocytopenia, anemia, and leukopenia) which got resolved after intravenous immunoglobulin (IVIG) administration. Both anti-Ro (SS-A) and anti-La (SS-B) antibodies were not detectable in his serum by immunodiffusion method while other such as RNP (nonspecific, including U1,U2,U3,...,U6), Sm and Scl-70 antibodies were all positive. This mother had all the above antibodies detectable in her serum. After excluding other possibilities, his pancytopenia was most likely to be attributed to neonatal lupus. We suggest that autoantibodies such as RNP and Sm antibodies may play an important role in the pathogenesis of thrombocytopenia of NLE.
自1981年抗Ro（SS-A）和/或抗La（SS-B）抗体被发现存在于新生儿狼疮（NLE）患儿及其母亲体内以来，后续研究表明NLE几乎普遍与这两种自身抗体中的一种或两种相关。据我们所知，有3例NLE患儿被报道抗Ro（SS-A）和抗La（SS-B）抗体呈阴性。我们报告1例患有系统性红斑狼疮（SLE）母亲所生的婴儿。该婴儿患有新生儿全血细胞减少症（血小板减少、贫血和白细胞减少），静脉注射免疫球蛋白（IVIG）后症状缓解。通过免疫扩散法在其血清中未检测到抗Ro（SS-A）和抗La（SS-B）抗体，而其他抗体如RNP（非特异性，包括U1、U2、U3、…、U6）、Sm和Scl-70抗体均为阳性。其母亲血清中可检测到所有上述抗体。排除其他可能性后，他的全血细胞减少症很可能归因于新生儿狼疮。我们认为，RNP和Sm等自身抗体可能在NLE血小板减少症的发病机制中起重要作用。
