SLE
Systemic lupus erythematosus (SLE) can have a profound effect on pregnancy outcome.
In what ways does SLE affect the babies?
SLE对妊娠的影响：
SLE通过哪些途径影响婴儿？
参考答案：
SLE通过哪些途径影响婴儿？
SLE女性体内还有抵抗多种细胞核或其他细胞成分的血清抗体，这些抗体的性质（类型和含量）因人而异。在部分SLE患者，存在的抗体与新生儿狼疮综合征（NLE）相关。对抗SSA/Ro或SSA/La核糖核蛋白复合体的母体抗体与NLE关系最密切[1，2]。
NLE最突出的两个临床表现是暂时的皮肤损害、永久的先天性心脏传导阻滞，但是也曾报道过出现自限性胆汁郁积性肝炎和白细胞减少[3，4]。该疾患的机制是被动自身免疫，母体抗体通过胎盘进入胎儿，导致胎儿组织破坏，从而在新生儿出现症状。有趣的是母体含有抗体的出生新生儿只有1-2%发生NLE，而且两种主要的症状表现也因时间的不同而异。妊娠18周到24周间常可发现心脏异常，而皮肤损害在出生后6周或更久[5]。心脏问题主要是房室传导阻滞，其中最常出现三度房室传导阻滞。虽然心脏结构上并无异常，但仍有10%以上的病例可能有相关性的心肌病变[6]。
细胞内抗原可能作用于细胞外母体抗体的机制尚未完全清楚。最近的证据表明，生理性凋亡可能促使细胞内抗原移位到细胞表面[7]；但也有研究表明，特定的抗体可能首先通过抑制靶细胞膜的钙内流，从而导致心律失常[8]。
In what ways does SLE affect the babies?
Women with systemic lupus erythematosus have serum antibodies directed against a variety of cell nuclear and other cellular components. The nature of these antibodies differs between patients, both in types and levels. In some patients with SLE, the antibodies present can be associated with a neonatal lupus syndrome (NLE). Maternal antibodies against the components of the SSA/Ro or SSA/La ribonucleoprotein complexes are those most closely linked to the neonatal lupus syndrome (1,2).
The two prominent clinical manifestations of this disorder include transient skin lesions, and permanent congenital heart block, although self-limited cholestatic hepatitis and cytopenia have also been reported (3,4). The mechanism for the disorder is passive auto-immunity, in which maternal antibodies are transported transplacentally, and result in tissue damage in the fetus, and subsequently in the newborn. The incidence of NLE is interesting in that it only occurs in a small subset, 1-2%, of the babies borne by mothers with the specific antibodies, and the two main manifestations differ in timing as well. Cardiac injury is usually detected between 18 and 24 weeks of gestation, while the skin findings develop 6 or more weeks after birth (5). The cardiac lesion is atrioventricular block, which can be of any degree, although third degree block is the most common. Although the heart is usually structurally normal, there may be an associated myopathy in up to 10% of cases (6).
The mechanism by which intracellular antigens are accessible to extracellular maternal antibodies has not yet been fully answered. Most recent evidence suggests that physiologic apoptosis serves as the mechanism to translocate intracellular antigens to the cell surface (7), while other reports have indicated that the specific antibodies may act first by inhibiting inward calcium fluxes across the target cell membranes, producing the arrythmia s (8).
References:
1. Buyon JP. Neonatal lupus syndromes. In: Lahita RG, ed. Systemic Lupus Erythematosus. 3rd ed. San Diego: Academic Press; 1999: 337-359.
2. Lee LA. Maternal autoantibodies and pregnancy-II: the neonatal lupus syndrome. In: Parke AL, ed. Bailliere's Clinical Rheumatology, Pregnancy and the Rheumatic Diseases. London: Bailliere Tindall; 1990:69-84.
3. Watson R, Kang JE, May M, et al. Thrombocytopenia in the neonatal lupus syndrome. Arch Dermatol 1988;124:560-563.
4. Laxer RM, Roberts EA, Gross KR, et al. Liver disease in neonatal lupus erythematosus. J. Pediatr 1990;116:238-242.
5. Buyon J. The heart and skin of neonatal lupus: Does maternal health matter? Am J Med. 2000;108:741-743.
6. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congential heart block, mortality, morbidity, and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol. 1998; 31:1658-1666.
7. Miranda-Careos ME, Tseng CE, Rashbaum W, et al. Accessibility of SSA/Ro,and SSB/La antigens to maternal autoantibodies in apoptotic human fetal cardiac myocytes. J Immunol. 1998;161:5061-5069.
8. Miranda-Careos ME, Boutjdir M, Tseng CE, et al. Induction of antibodies reactive with SSA/Ro-SSB/La and development of congenital heart block in a murine model. J Immunol. 1998;161:5886-5892
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