新生兒紅斑性狼瘡為一種罕見的疾病,起因為患有系統紅斑性狼瘡的母親,她的自體抗體經由胎盤傳給胎兒,而導致新生兒皮膚、心臟及血液的一些症狀。從1981年起,新生兒紅斑性狼瘡被報告和Ro(SSA)或La(SSB)抗體有密切的關係之後,陸續的臨床研究報告顯示幾乎所有的病例皆至少具有Ro或La抗體其中之一。到目前為止,據我們所知,文獻上只有三例新生兒紅斑性狼瘡被報告不具有Ro或La抗體。我們報告一32週新生兒患嬰,其母親為一位系統紅斑性狼瘡之患者,血清中具有Ro,La,RNP及Sm等抗體。這名新生兒主要臨床表現為出生後到十天大有血球過低的現象(包括紅血球,白血球及血小板)。在審慎考慮其它可能性之後,我們認為其血球過低可能起因於新生兒紅斑性狼瘡之活性。在使用免疫球蛋白之後,這名新生兒的血球逐漸回復正常。這名患嬰血中並無Ro或La抗體但卻具有RNP及Sm抗體。由上述發現我們認為Ro或La抗體以外的自體抗體可以引起新生兒紅斑性狼瘡,特別是血液中的表現可能是由RNP,Sm或者其它自體抗體所造成。去偵測其它我們未知卻可引起新生兒紅斑性狼瘡的自體抗體是我們應努力的方向。
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.