I. Miniaoui, MD, N. Morel, MD, K. Lévesque, MD, G. Guettrot-Imbert, MD, V. Le Guern, MD, L. Mouthon, MD, PhD, M. Jallouli, MD, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares d'Ile de France, Paris.
A. Maltret, MD, J. Le Bidois, MD, D. Bonnet, MD, PhD, AP-HP, Centre de référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker-Enfants malades, Université de Paris, Paris.
J Rheumatol. 2022 Oct;49(10):1124-1130. doi: 10.3899/jrheum.210703. Epub 2022 May 2.
Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).
This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.
We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia ( < 0.001), dry syndrome ( = 0.01), and parotid swelling ( = 0.05).
One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required.
母体抗 SSA 和抗 SSB 抗体经胎盘传递，可能与母体自身免疫性疾病相关，可导致新生儿狼疮综合征。鉴于此背景下的数据匮乏，我们报告了患有先天性心脏传导阻滞（CHB）的患儿的母亲的短期和长期结局。
这项回顾性研究纳入了抗 SSA/SSB 抗体阳性的胎儿患有高度 CHB 的母亲，并重点关注了她们在妊娠前、CHB 诊断时以及此后的健康状况。
我们分析了 215 名至少有一次 CHB 妊娠的女性。在诊断出该疾病之前，仅有 52 名（24%）母亲被诊断患有自身免疫性疾病，主要为系统性红斑狼疮（SLE；n = 26，12%）和干燥综合征（SS；n = 16，7%）。在本次妊娠期间，又有 6 名母亲被诊断患有自身免疫性疾病。在分娩时无此类诊断的 157 名母亲（73%）中，有 77 名（49%）在中位随访 11 年后（范围：21 天至 54 年）被诊断出患有自身免疫性疾病。随访结束时，135 名女性（63%）被诊断出患有自身免疫性疾病，主要为 SLE（n = 54，25%）和 SS（n = 72，33%）。3 名 SLE 患者出现肾脏受累，仅有 6 名（3%）患者在任何时候需要使用免疫抑制药物。预测自身免疫性疾病发展的最佳症状是关节痛和肌痛（<0.001）、干燥综合征（=0.01）和腮腺肿胀（=0.05）。
在胎儿 CHB 诊断时，四分之一的患者被诊断出患有自身免疫性疾病。在随访期间，近一半最初未被诊断出的患者出现了进展，且大多数患者没有严重表现。很少见到狼疮肾炎等严重疾病，也很少需要使用免疫抑制药物。
