Cardiology Service, Hospital General de Occidente, Ministry of Health.
National Institutes of Health, Ministry of Health.
Arch Cardiol Mex. 2024;94(1):33-38. doi: 10.24875/ACM.22000233.
Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement.
To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up.
From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL.
Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations.
CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.
新生儿狼疮（NL）极为罕见，是由于母体 IgG 自身抗体经胎盘进入胎儿循环，从而导致先天性完全性房室传导阻滞（CCAB）、永久性皮肤损伤和肝脏受累。这些自身抗体针对 Ro、La 和/或 RNP 蛋白。
了解 CCAB 患者中 NL 的患病率以及长期随访中的临床病程。
1992 年 1 月至 2017 年 12 月，纳入 CCAB 患者。母亲血清中抗 SSA/Ro 和抗 SSB/La 抗核抗体的存在证实了 NL 的存在。
共纳入 8 例患者，随访时间为 10±6 年；NL 的结论为 62.5%；2 例为男性。其中 1 例在宫内诊断，2 例在出生时诊断，并植入了起搏器，1 例在 12 岁时，另 1 例在 15 岁时。另外 2 例分别在 18 岁和 26 岁时诊断，分别在 8 年和 5 年后植入了永久性起搏器。在 1 例新生儿中，仅随访 1 年就未植入起搏器。分娩时，60%的母亲没有风湿病，共有 19 个孩子，均无 NL 表现。
CCAB 罕见，常与母体自身免疫性疾病相关，几乎所有患者在其一生中的某个时刻都需要植入永久性起搏器。
