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未婚病人所生小孩或许有该病症的新生儿显出： 1、该病症的主要病因是什么？ 2、该病症的临床步骤？ 参考答案： 1、该病症的主要病因是什么？ SLE及无关紊乱的未婚病人血清内可含多种不同突变，这些抗SSA/Ro或SSB/La突变或许具有新生儿狼疮（NLE）突变[1]。两种主要病因都有永久性先天性肾脏传导阻滞和则会脸部危及。一些新生儿可只有一种显出，也可两种都有。通过对这些新生儿的检查，深入研究医务人员希望发掘出是不是是什么环境因素导致了NLE的一种或多种显出。近期的一项深入研究[2]，调查了1981年到1997年过后国家不须向有NLE脸部危及的小孩，结果发掘出，261名病人中，57名有溃疡显出但没先天性肾脏传导阻滞。不须向的小孩没种族相似之处。所有父母含SSA/Ro、SSB/La或U1RNP突变，但最后一个突变只发掘出实际上于一个母体，而不含其他突变。SLE的父母格外或许生下有脸部显出的小孩，但这些父母总的健康状况与那些于其先天性肾脏传导阻滞新生儿的父母并无相似之处。 2、该病症的临床步骤？ 这些受接种的新生儿最常常显现出孤立皮损（44/57），其余则可有肝脏或皮肤病科显出。显现出溃疡的大约时间段约为6周（0-20周），其中1/4新生儿在出生时即有显出。溃疡持续1.5到52周，大约17周，常常有下巴显出。一般皮肤病有腿部脸部显出，1/4显现出四肢或躯干脸部显出。溃疡有红斑，主要为环形，常常无内侧，但近1/3有鳞屑显出。光敏感性为其显眼适应性，这在NLE的其他深入研究中也曾提到[3]。 57名中的34名运用于均匀分布皮质激素治疗法，所有病人溃疡遗忘，且通常常无后遗症。其余有NLE显出的病人（30%有先天性肾脏传导阻滞），65%分割有脸部显出。该数值优于既往的路透社值8-25%[4，5]。对接种的新生儿顺利完成了大约77个月的辨别，57名新生儿中有4名显现出自身免疫性结核病，都有野田氏甲状腺炎、类风湿性关节炎和雷诺氏自然现象。剩93%的新生儿辨别随访中无异常常。 虽然这些显出最初是不得不的，且可治愈，但是在其后的妊娠中工业发展为先天性肾脏传导阻滞的有或许很高。肾脏传导阻滞并不是不得不的，对于这种状况的病人格外不须慎重。 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. 编者：ache 编者： ache
