新生兒紅斑性狼瘡之臨床表徵和實驗分析

Clinical spectrum and laboratory characteristics of neonatal lupus erythematosus in a single institute

新生兒紅斑性狼瘡主要是經由母體胎盤傳送的Anti-Ro/SSA、Anti-La/SSB或者是Anti-U1RNP抗體所造成的被動性自體免疫反應。這些抗體在胎兒的體內數個月內均可被測到。其臨床上常見的表徵可以有皮膚紅斑和心臟房室阻斷。至於血液疾病，肝臟方面或神經系統的問題常常被忽略。我們回顧1984年至2003年，共有12個病例。我們就臨床表徵、實驗數據和母親的關係予以分析。男性和女性的比例為1.4:1。心臟房室阻斷有五個病人，皮膚紅斑有七個病人，血小板低下有兩個病人，貧血有兩個病人，肝功能異常有三個病人，有一個病人產生持續性的黃疸，一個病人有神經系統的問題而產生局部性的抽搐。Anti-Ro/ss的抗體在12個病人的身上均可發現，陽性ANA指數有10個病人，而Anti-La/SSB抗體在5個病人身上可發現，這5個病人均有皮膚紅斑卻無心臟房室阻斷。在母親方面，12個中有8個為紅斑性狼瘡，另外4個在臨床沒有症狀，這4個沒有症狀的母親有1個之後發展為紅斑性狼瘡，而新生兒紅斑性狼瘡的母親也容易有流產的病史。因此我們認為產前超音波對於早期診斷心臟房室阻斷是很重要的。對於那些血中含有高濃度Anti-Ro/SSA、Anti-La/SSB 的母親，包括了臨床上沒有症狀的媽媽，我們都應小心追蹤這些嬰兒至少6個月以上，因為這些嬰兒可能在出生數個星期之後才可能有臨床症狀。總而言之，對於有臨床症狀的病人我們都應該考慮到其他的表徵。

Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants. Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003. Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti -SSB/La antibodies were not present in any of the infants with congenital heart block. Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.