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Journal of Peking University (Health Sciences) ›› 2023, Vol. 55 ›› Issue (6): 1053-1057. doi: 10.19723/j.issn.1671-167X.2023.06.015

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Clinical characteristics of fetal cardiac disease in patients with anti-SSA antibody positive

Yu-fei LI1,Ya-ni YAN2,Jia-yang JIN1,Chun LI1,*(),Qiu-yan PEI2,*()   

  1. 1. Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China
    2. Department of Obstetrics & Gynecology, Peking University People' s Hospital, Beijing 100044, China
  • Received:2023-08-22 Online:2023-12-18 Published:2023-12-11
  • Contact: Chun LI,Qiu-yan PEI E-mail:fiona_leechun@163.com;pqypei@126.com
  • Supported by:
    China lnternational Medical Foundation(Z-2018-40-2101);Peking University People' s Hospital Scientific Research Development Funds(RD 2022-66)

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Abstract:

Objective: To investigate the clinical manifestations and laboratory indicators of anti-Sjögren's-syndrome-related antigen A (SSA) antibody associated fetal cardiac disease. Methods: Pregnant women hospitalized at Peking University People's Hospital from January 2013 to July 2023 were included. Eleven patients with anti-SSA antibody positive were eventually diagnosed with fetal cardiac di-sease. And patients with anti-SSA antibody positive without fetal cardiac disease were selected as controls. Clinical manifestations, laboratory indications and drug usage were compared between the two groups. Results: Among these 11 patients, congenital heart block was confirmed in seven, which was the most common manifestations of fetal cardiac malformation. The proportion of the patients diagnosed with autoimmune disease before pregnancy in fetal cardiac malformation group was significantly lower than that in the control group (P=0.032), while most of the patients in the fetal cardiac malformation group received immune-related examinations for the first time because of this time's fetal cardiac diagnosis. While most of the patients in the control group received routine examinations because of autoimmune diseases diagnosed before pregnancy. During pregnancy, the white blood cell level [(9.29±2.58)×109/L vs. (7.10±1.90×109/L, t=3.052, P=0.004], erythrocyte sedimentation rate [(49.50 (48.00, 51.00) mm/h vs. 23.00 (15.00, 30.25) mm/h, Z=-2.251, P=0.024], IgA level [3.46 (2.30, 5.06) g/L vs. 2.13 (1.77, 2.77) g/L, Z=-2.181, P=0.029], and antinuclear antibody (ANA) titers [1∶320 (1∶160, 1∶320) vs. 1∶80 (1∶40, 1∶160), Z=-3.022, P=0.003] were significantly higher in fetal cardiac malformation group than in the control group. The proportion of positive anti-SSB antibody during pregnancy did not show a statistically significant difference between the two groups (37.5% vs. 7.7%, P=0.053). There was no significant difference in hydroxychloroquine dosage and initiation time between the two groups. The dosage of prednisone in the second and third trimesters was significantly higher in the cardiac malformation group than that in the control group, but there was no significant difference in the first trimester. Conclusion: Fetal cardiac disease is rare in pregnant women with anti-SSA antibody. White blood cell, erythrocyte sedimentation rate, IgA, the titer of ANA positivity were higher in the fetal heart disease group during pregnancy. Since congenital heart block is difficult to reverse, its prevention and monitoring are more important than remedial treatment.

Key words: Anti-Sjögren's-syndrome-related antigen A antibody, Congenital heart defects, Heart block, Pregnancy, Risk factors

CLC Number: 

  • R593.2

Table 1

The comparison of clinical symptoms and complications during pregnancy between the cardial malformation and the control group"

Parameters Cardiac malformation (n=11) Control (n=44) P
Clinical feature, n(%)
  History of miscarriage 5 (45.5) 22 (50.0) > 0.999
  Leukopenia during pregnancy 0 7 (15.9) 0.323
  Thrombocytopenia during pregnancy 2 (18.2) 10 (22.7) > 0.999
  Diagnosis of autoimmune disease before pregnancy 6 (54.5) 38 (86.4) 0.032
Complicating disease, n(%)
  Hashimoto thyroiditis 2 (18.2) 2 (4.5) 0.175
  Hyperthyroidism 0 0 > 0.999
  Hypothyroidism 1 (9.1) 8 (18.2) 0.669
  Gestational diabetes mellitus 1 (9.1) 3 (6.8) > 0.999
  Ovarian tumor 0 2 (4.5) > 0.999

Table 2

The comparison of laboratory parameters between the cardiac malformation and the control group"

Parameters Cardiac malformation (n=11) Control (n=44) Z/t/χ2 P
WBC during pregnancy/(×109/L) 9.29±2.58 7.10±1.90 3.052 0.004
PLT during pregnancy/(×109/L) 202.20±79.05 171.51±56.68 1.428 0.159
ESR during pregnancy/(mm/h) 49.50 (48.00, 51.00) 23.00 (15.00, 30.25) -2.251 0.024
IgA during pregnancy/(g/L) 3.46 (2.30, 5.06) 2.13 (1.77, 2.77) -2.181 0.029
ANA positive during pregnancy 10/10 (100.0) 38/40 (95.0) > 0.999
ANA titer during pregnancy 1∶320 (1∶160, 1∶320) 1∶80 (1∶40, 1∶160) -3.022 0.003
Anti-SSB positive during pregnancy 3/8 (37.5) 3/39 (7.7) 0.053

Table 3

The comparison of medication between the cardiac malformation and the control group"

Parameters Cardiac malformation (n=11) Control (n=44) Z/t/χ2 P
HCQ
  Use of HCQ during pregnancy, n(%) 10 (90.9) 35 (79.5) 0.667
  Use of HCQ during the first trimester, n(%) 6 (54.5) 33 (75.0) 0.266
  HCQ dose in the first trimester/mg, M (P25, P75) 200 (0, 200) 200 (25, 200) -1.117 0.264
  HCQ dose in the second trimester/mg, M (P25, P75) 200 (200, 200) 200 (100, 200) -0.555 0.579
  HCQ dose in the third trimester/mg, M (P25, P75) 200 (200, 200) 200 (125, 200) -1.098 0.272
Prednisone
  Use of prednisone during pregnancy, n(%) 9 (81.8) 16 (36.4) 0.015
  Prednisone dose in the first trimester/mg, M (P25, P75) 0 (0, 10.00) 0 (0, 5.00) -0.593 0.553
  Prednisone dose in the second trimester/mg, M (P25, P75) 10.00 (0, 20.00) 0 (0, 5.00) -2.924 0.003
  Prednisone dose in the third trimester/mg, M (P25, P75) 10.00 (5.00, 26.70) 0 (0, 5.00) -3.347 0.001
1 Yuan T , Shuo Y , Qi L , et al. Pregnancy-related complications in systemic lupus erythematosus[J]. J Autoimmun, 2022, 132, 102864.
doi: 10.1016/j.jaut.2022.102864
2 Merz WM , Fischer-Betz R , Hellwig K , et al. Pregnancy and autoimmune disease[J]. Dtsch Arztebl Int, 2022, 119 (9): 145- 156.
3 Fausta B , Camilla B , Irene MD , et al. Impact of pregnancy on progression of preclinical autoimmune disorders: A prospective cohort study[J]. Rheumatology (Oxford), 2022, 62 (9): 2971- 2978.
4 Marder W , Littlejohn EA , Somers EC . Pregnancy and autoimmune connective tissue disease[J]. Best Pract Res Clin Rheumatol, 2016, 30 (1): 63- 80.
doi: 10.1016/j.berh.2016.05.002
5 Fischer-Betz R , Specker C . Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome[J]. Best Pract Res Clin Rheumatol, 2017, 31 (3): 397- 414.
doi: 10.1016/j.berh.2017.09.011
6 李珊, 李明, 张旭. 19例新生儿红斑狼疮的心血管表现[J]. 广州医药, 2022, 53 (6): 48- 51.
7 Julkunen H , Eronen M . Long-term outcome of mothers of children with isolated heart block in Finland[J]. Arthritis Rheum, 2001, 44 (3): 647- 652.
doi: 10.1002/1529-0131(200103)44:3<647::AID-ANR113>3.0.CO;2-I
8 Izmirly MP , Saxena A , Kim YM , et al. Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus[J]. Circulation, 2011, 124 (18): 1927- 1935.
doi: 10.1161/CIRCULATIONAHA.111.033894
9 Saxena A , Izmirly PM , Bomar RP , et al. Factors associated with long-term cardiac dysfunction in neonatal lupus[J]. Ann Rheum Dis, 2020, 79 (2): 217- 224.
doi: 10.1136/annrheumdis-2019-215900
10 Habib SM , Vermeer MH . A baby with red plaques on the face and a first-degree heart block: Neonatal lupus[J]. Lancet, 2020, 396 (10260): 1432.
doi: 10.1016/S0140-6736(20)32176-0
11 Wainwright B , Bhan R , Trad C , et al. Autoimmune-mediated congenital heart block[J]. Best Pract Res Clin Obstet Gynaecol, 2020, 64, 41- 51.
doi: 10.1016/j.bpobgyn.2019.09.001
12 Brito-Zerón P , Izmirly PM , Ramos-Casals M , et al. The clinical spectrum of autoimmune congenital heart block[J]. Nat Rev Rheumatol, 2015, 11 (5): 301- 312.
doi: 10.1038/nrrheum.2015.29
13 Aurélie A , Vijole D , Jeongsook P , et al. Anti-Ro52 monoclonal antibodies specific for amino acid 200-239, but not other Ro52 epitopes, induce congenital heart block in a rat model[J]. Ann Rheum Dis, 2012, 71 (3): 448- 454.
doi: 10.1136/annrheumdis-2011-200414
14 Llanos C , Friedman DM , Saxena A , et al. Anatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus[J]. Rheumatology (Oxford), 2012, 51 (6): 1086- 1092.
doi: 10.1093/rheumatology/ker515
15 Cuneo BF , Strasburger JF , Niksch A , et al. An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease[J]. J Matern Fetal Neonatal Med, 2009, 22 (3): 233- 238.
doi: 10.1080/14767050802488220
16 Tunks DR , Clowse EM , Miller GS , et al. Maternal autoantibody levels in congenital heart block and potential prophylaxis with antiinflammatory agents[J]. Am J Obstet Gynecol, 2013, 208 (1): 64.e1- 64.e7.
doi: 10.1016/j.ajog.2012.09.020
17 Jaeggi E , Laskin C , Hamilton R , et al. The importance of the level of maternal anti-Ro/SSA antibodies as a prognostic marker of the development of cardiac neonatal lupus erythematosus a prospective study of 186 antibody-exposed fetuses and infants[J]. J Am Coll Cardiol, 2010, 55 (24): 2778- 2784.
doi: 10.1016/j.jacc.2010.02.042
18 Shinohara K , Miyagawa S , Fujita T , et al. Neonatal lupus erythematosus: Results of maternal corticosteroid therapy[J]. Obstet Gynecol, 1999, 93 (6): 952- 957.
19 刘蕾, 华益民, 周开宇. 新生儿狼疮综合征诊疗研究进展[J]. 中国循证儿科杂志, 2018, 13 (3): 231- 235.
20 Izmirly P , Kim M , Friedman MD , et al. Hydroxychloroquine to prevent recurrent congenital heart block in fetuses of anti-SSA/Ro-positive mothers[J]. J Am Coll Cardiol, 2020, 76 (3): 292- 302.
doi: 10.1016/j.jacc.2020.05.045
21 Julie B , Nathalie C , Adey B , et al. Effect of in utero hydroxychloroquine exposure on the development of cutaneous neonatal lupus erythematosus[J]. Ann Rheum Dis, 2018, 77 (12): 1742- 1749.
doi: 10.1136/annrheumdis-2018-213718
22 Strasburger FJ , Wacker-Gussmann A . Congenital heart block in subsequent pregnancies of SSA/Ro-positive mothers[J]. J Am Coll Cardiol, 2020, 76 (3): 303- 305.
doi: 10.1016/j.jacc.2020.05.052
23 Friedman DM , Kim MY , Copel JA , et al. Utility of cardiac monitoring in fetuses at risk for congenital heart block: The PR Interval and Dexamethasone Evaluation (PRIDE) prospective study[J]. Circulation, 2008, 117 (4): 485- 493.
24 Gleicher N , Elkayam U . Preventing congenital neonatal heart block in offspring of mothers with anti-SSA/Ro and SSB/La antibodies: A review of published literature and registered clinical trials[J]. Autoimmun Rev, 2013, 12 (11): 1039- 1045.
25 张利, 米荣, 王晓颖, 等. 静脉注射人免疫球蛋白治疗45例新生儿红斑狼疮的疗效[J]. 中国新药与临床杂志, 2023, 42 (6): 378- 382.
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