Press J, Uziel Y, Laxer R M, Luy L, Hamilton R M, Silverman E D
Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Canada.
Am J Med. 1996 Mar;100(3):328-32. doi: 10.1016/S0002-9343(97)89492-2.
To determine the health of mothers of offspring with complete congenital heart block (CHB) both at the time of delivery of the affected child and in the long-term, and the percentage of mothers and children with CHB who had anti-SSA/Ro and/or SSB/La antibodies.
Sixty-four mothers of 64 children with CHB (seen between 1964 and 1993) were identified through the Cardiology database of The Hospital for Sick Children, Toronto, Canada. Medical information from these of children with CHB was evaluated. Data were obtained from the mothers by mailed questionnaire, telephone interview, and/or from the attending physicians. The presence of anti-Ro antibodies and anti-La antibodies were evaluated by ELISA assay.
The mean age at the time of delivery of the first child with CHB was 28 +/- 6 years. At the time of delivery 42 (66%) mothers were healthy, 2 (3%) had systemic lupus erythematosus (SLE), 2 (3%) had linear scleroderma, 2 (3%) had rheumatoid arthritis; 3 (5%) had a history of rheumatic fever (but were otherwise well), 1 (2%) had Sjogren's syndrome (SS), and 12 (19%) had an undifferentiated autoimmune syndrome (UAS) (arthralgia, myalgia, photosensitivity, skin vasculitis, Raynaud's phenomenon). The mean time to follow-up from deliver to study was 121 +/- 88 months. The mean maternal age at study was 38 +/- 9 years. Three of 12 mothers who initially had a UAS progressed to SLE (average follow-up time of 80 months, median 96), and 2 developed SS (with average follow-up time 140 months, median 132) and 1 went into remission. The mean follow-up time for the other mothers who did not develop an autoimmune disease was 150 +/- 102 months. Thirty-six of the 42 initially healthy mothers remained well. One mother developed SLE; 1 developed hyperthyroidism; 1 developed anky-losing spondylitis; and 3 developed an UAS. The mean follow-up time of the 36 mothers who remained healthy was similar (123 +/- 97 months) to the 6 initial healthy mothers who developed an autoimmune disease (121 +/- 36 months). Anti-Ro and/or anti-La antibodies were positive in 32 of 53 (60%) mothers tested. Fourteen of the 53 mothers were symptomatic at the time of delivery and 39 were asymptomatic. Anti-Ro and/or anti-La antibodies were positive in 12 of 13 mothers tested at the time of delivery.
The long-term maternal outcome in our cohort was very good as most of the initially healthy mothers remained well at follow-up. Twenty-five percent of the mothers with a UAS and only 2% of the initially healthy mothers developed SLE. The development of an autoimmune disease in an asymptomatic mother identified by the birth of a child with CHB was less common in our study than in previous studies. However, close follow-up of mothers with UAS is warranted.
确定患有完全性先天性心脏传导阻滞（CHB）患儿的母亲在分娩时及长期的健康状况，以及患有CHB的母亲和儿童中抗SSA/Ro和/或SSB/La抗体阳性的比例。
通过加拿大多伦多病童医院的心脏病学数据库，确定了64名患有CHB儿童的64位母亲（于1964年至1993年间就诊）。对这些患有CHB儿童的医疗信息进行了评估。通过邮寄问卷、电话访谈和/或主治医生获取母亲的数据。采用酶联免疫吸附测定法评估抗Ro抗体和抗La抗体的存在情况。
首位患有CHB儿童出生时母亲的平均年龄为28±6岁。分娩时，42名（66%）母亲健康，2名（3%）患有系统性红斑狼疮（SLE），2名（3%）患有线状硬皮病，2名（3%）患有类风湿关节炎；3名（5%）有风湿热病史（但其他方面健康），1名（2%）患有干燥综合征（SS），12名（19%）患有未分化自身免疫综合征（UAS）（关节痛、肌痛、光敏性、皮肤血管炎、雷诺现象）。从分娩到研究的平均随访时间为121±88个月。研究时母亲的平均年龄为38±9岁。最初患有UAS的12名母亲中有3名进展为SLE（平均随访时间80个月，中位数96个月），2名发展为SS（平均随访时间140个月，中位数132个月），1名病情缓解。其他未发展为自身免疫性疾病的母亲的平均随访时间为150±102个月。42名最初健康的母亲中有36名仍保持健康。1名母亲患SLE；1名患甲状腺功能亢进；1名患强直性脊柱炎；3名发展为UAS。36名保持健康的母亲的平均随访时间（123±97个月）与6名发展为自身免疫性疾病的最初健康母亲的平均随访时间（121±36个月）相似。在接受检测的53名母亲中，32名（60%）抗Ro和/或抗La抗体呈阳性。53名母亲中有14名在分娩时出现症状，39名无症状。在分娩时接受检测的13名母亲中，12名抗Ro和/或抗La抗体呈阳性。
我们队列中的母亲长期预后非常好，因为大多数最初健康的母亲在随访时仍保持健康。患有UAS的母亲中有25%，而最初健康的母亲中只有2%发展为SLE。在我们的研究中，因CHB患儿出生而被确定为无症状的母亲发展为自身免疫性疾病的情况比以往研究中少见。然而，对患有UAS的母亲进行密切随访是必要的。
