SLE
We continue our discussion of SLE today. Babies born to mothers with systemic lupus erythematosus (SLE) may have a neonatal form of the disease.
1. What are the major clinical manifestations of this disease?
2. What is the clinical course of this disease?
SLE女性患者所生婴儿可能有该疾患的新生儿表现：
1、该疾患的主要临床表现是什么？
2、该疾患的临床过程？
参考答案：
1、该疾患的主要临床表现是什么？
SLE及相关紊乱的女性患者血清内可含有多种不同抗体，这些抗SSA/Ro或SSB/La抗体可能具有新生儿狼疮（NLE）抗体[1]。两种主要临床表现包括永久性先天性心脏传导阻滞和暂时性皮肤损害。一些新生儿可只有一种表现，也可两种都有。通过对这些患儿的检查，研究人员希望发现究竟是什么因素导致了NLE的一种或多种表现。最近的一项研究[2]，调查了1981年到1997年期间国家登记处有NLE皮肤损害的婴儿，结果发现，261名患者中，57名有皮疹表现但没有先天性心脏传导阻滞。登记处的婴儿没有种族差异。所有母亲含有SSA/Ro、SSB/La或U1RNP抗体，但最后一个抗体只发现存在于一个母体，而不含有其他抗体。SLE的母亲更可能生下有皮肤表现的婴儿，但这些母亲总的健康状况与那些生有先天性心脏传导阻滞患儿的母亲并无差异。
2、该疾患的临床过程？
这些受感染的患儿最常出现孤立皮损（44/57），其余则可有肝脏或血液学表现。出现皮疹的平均时间约为6周（0-20周），其中1/4患儿在出生时即有表现。皮疹持续1.5到52周，平均17周，常有面部表现。一般小儿有头部皮肤表现，1/4出现四肢或颈部皮肤表现。皮疹有红斑，主要为环形，常无隆起，但近1/3有鳞屑表现。光敏感性为其突出特性，这在NLE的其他研究中也曾提到[3]。
57名中的34名采用局部皮质激素治疗，所有患者皮疹消失，且通常无后遗症。其余有NLE表现的患者（30%有先天性心脏传导阻滞），65%合并有皮肤表现。该数值高于既往的报道值8-25%[4，5]。
对感染的患儿进行了平均77个月的观察，57名患儿中有4名出现自身免疫性疾病，包括桥本氏甲状腺炎、类风湿性关节炎和雷诺氏现象。剩余93%的患儿观察随访中无异常。
虽然这些表现最初是暂时的，且可治愈，但是在后来的妊娠中发展为先天性心脏传导阻滞的几率很高。心脏传导阻滞并不是暂时的，对于这种状况的诊断更须慎重。
What are the major clinical manifestations of this disease?
While woman with SLE and related disorders may have a number of different antibodies in their serum, those with anti SSA/Ro or anti-SSB/La may have a baby with neonatal lupus (NLE) (1). The two major clinical manifestations include permanent congenital heart block and transient skin lesions. Some babies get one manifestation while some may have both. By examining the babies with each finding, investigators have tried to understand what factors might predispose the development of one or the other form of NLE. A recent study (2) examined infants identified through a large national registry as having the cutaneous findings of NLE, during the years 1981-1997. Of 261 babies in the registry, 57 had skin rash in the absence of congenital heart block. There was no difference in the ethnicity of these babies from the rest of the registry. All of the mothers had serum antibodies to SSA/Ro, SSB/La or U1RNP, although this last antibody was only present in one mother without at least one of the other two. Mothers with SLE were more likely to have babies with cutaneous findings, but the overall health status of these mothers was no different from that of the mothers whose babies had congenital heart block.
What is the clinical course of this disease?
The affected babies mostly had isolated skin lesions (44/57). The others had hepatic and/or hematologic involvement as well. The mean age of detection of the rash was 6 weeks (0-20 weeks), with about a quarter of the children presenting at birth. The rash lasted between 1.5 and 52 weeks, with a mean length of 17 weeks, and generally included facial findings. Half of the children had lesions on their scalp, and about a quarter had lesions on the extremities or neck. The rash itself was erythematous and primarily annular, usually without a raised border, but almost 1/3 had a fine scale. Photosensitivity was a prominent feature, as has been noted in other studies of NLE (3).
Thirty-four of 57 were treated with topical corticosteroids, and in all of the patients the rash resolved usually without sequelae. Sixty-five percent of subsequent siblings had manifestations of NLE, including 30% with congenital heart block, often in association with a skin rash. This rate is higher than previous reported values of 8-25% (4,5).
The affected children were followed for a mean period of 77 months. Four of fifty-seven developed autoimmune disease, including Hashimoto's thyroiditis, rheumatoid arthritis, and Raynaud's phenomenon. The remaining 93% of the children were well at followup.
This manifestation is thus primarily transient and treatable, although the risk of "cross-over" to congenital heart block in subsequent pregnancies is significant. Heart block is not transient, and the prognosis for this condition is much more guarded.
References:
1. Buyon JP. Neonatal lupus syndromes. In: Lahita RG, ed. Systemic Lupus Erythematosus. 3rd ed. San Diego: Academic Press; 1999: 337-359.
2. Neiman AR, Lee LA, Weston WL, Buyon JP. Cutaneous manifestations of neonatal lupus without heart block: Characteristics of mothers and children enrolled in a national registry. J Pediatr 2000;137:674-680.
3. Weston WL, Morelli JG, Lee LA. The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus. J Am Acad Dermatol. 1999;40:675-681.
4. McCune AB,Weston WL, Lee LA. Maternal and fetal outcome in neonatal lupus erythematosus. Ann Intern Med 1987;106:518-523.
5. Julkunen H, Kaaja R, Wallgren E, Teramo K. Isolated congenital heart block: fetal and infant outcome and familial incidence of heart block. Obstet Gynecol 1993;82:11-16.
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