Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus
Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus
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DOI：
10.1136/ard.2008.088054
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发表时间：
2009-06-01
影响因子：
27.4
通讯作者：
Buyon, J. P.
中科院分区：
文献类型：
--
作者：
Rivera, T. L.;Izmirly, P. M.;Buyon, J. P.
目的：评估无症状和少症状的新生儿狼疮（NL）的母亲的自身免疫性疾病进展。方法：从病历中获得参加NL研究登记处（RRNL）的母亲的临床信息。基因分型进行了2308 A/G肿瘤坏死因子（TNF）α，869 T/C转化生长因子（TGF）β和-889 C/T白细胞介素（IL）1 α。结果：321名母亲参加，229至少有6个月的后续行动。在NL婴儿出生时无症状的51名母亲中，26名进展：12名发展为少未分化自身免疫综合征（少UAS），2名多UAS，7名SS，4名SLE和1名SLE/SS。出现任何症状的中位时间为3.15年。在NL孩子出生时被归类为少UAS的37位母亲中，16位进展：5位发展为多UAS，6位Sjogren综合征（SS），4位系统性红斑狼疮（SLE）和1位SLE/SS。在1年内登记的少UAS母亲中，进展的中位时间为6.7年。4名母亲发生狼疮性肾炎（2名无症状，2名少UAS）。无症状母亲10年内发生SLE的概率为18.6%，发生可能/明确SS的概率为27.9%。NL表现不能预测无症状母亲的疾病进展。抗干燥综合征A抗原（SSA/）Ro和抗干燥综合征B抗原（SS B）/La的母亲发生自身免疫性疾病的可能性几乎是仅抗SSA/Ro的母亲的两倍。只有TGFbT/T显着较高的SLE母亲相比，无症状的母亲（p = 0.03）。结论：无症状的NL母亲的持续随访是必要的，因为近一半的进展，虽然很少发展SLE。虽然抗SSB/La抗体可能是疾病进展的危险因素，但需要进一步的工作来确定其他健康女性的可靠生物标志物，这些女性的抗SSA/Ro抗体仅因NL儿童而被确定。
Objective: To evaluate autoimmune disease progression in asymptomatic and pauci-symptomatic mothers of children with neonatal lupus (NL).Methods: Clinical information on mothers enrolled in the Research Registry for NL (RRNL) was obtained from medical records. Genotyping was performed for 2308A/G tumour necrosis factor (TNF)alpha, 869T/C transforming growth factor (TGF)beta and -889C/T interleukin (IL)1 alpha.Results: Of the 321 mothers enrolled, 229 had at least 6 months of follow-up. Of the 51 mothers who were asymptomatic at the NL child's birth, 26 progressed: 12 developed pauci-undifferentiated autoimmune syndrome (pauci-UAS), 2 poly-UAS, 7 SS, 4 SLE and 1 SLE/SS. The median time to develop any symptom was 3.15 years. Of the 37 mothers classified as pauci-UAS at the NL child's birth, 16 progressed: 5 developed poly-UAS, 6 Sjogren syndrome (SS), 4 systemic lupus erythematosus (SLE) and 1 SLE/SS. Of the pauci-UAS mothers enrolled within 1 year, the median time to progression was 6.7 years. Four mothers developed lupus nephritis (two asymptomatic, two pauci-UAS). The probability of an asymptomatic mother developing SLE by 10 years was 18.6%, and developing probable/definite SS was 27.9%. NL manifestations did not predict disease progression in an asymptomatic mother. Mothers with anti-Sjogren syndrome A antigen (SSA/)Ro and anti-Sjogren syndrome B antigen (SSB)/La were nearly twice as likely to develop an autoimmune disease as mothers with anti-SSA/Ro only. Only TGFbT/T was significantly higher in SLE mothers compared to asymptomatic mothers (p = 0.03).Conclusions: Continued follow-up of asymptomatic NL mothers is warranted since nearly half progress, albeit few develop SLE. While the anti-SSB/La antibodies may be a risk factor for progression, further work is needed to determine reliable biomarkers in otherwise healthy women with anti-SSA/Ro antibodies identified solely because of an NL child.