Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Outcome of pregnant mothers with systemic lupus erythematosus (focusing on congenital heart block)

전신성 홍반성 루푸스 산모의 출산아의 임상적 고찰 (완전 방실 블록을 중심으로)

  • Baek, Hey Sung (Department of Pediatrics, College of Medicine, Hanyang University) ;
  • Choi, Jae Hyung (Department of Pediatrics, College of Medicine, Hanyang University) ;
  • Kim, Nam Su (Department of Pediatrics, College of Medicine, Hanyang University) ;
  • Kim, Chang Ryul (Department of Pediatrics, College of Medicine, Hanyang University) ;
  • Moon, Su Ji (Department of Pediatrics, College of Medicine, Hanyang University)
  • 백혜성 (한양대학교 의과대학 소아과학교실) ;
  • 최재형 (한양대학교 의과대학 소아과학교실) ;
  • 김남수 (한양대학교 의과대학 소아과학교실) ;
  • 김창렬 (한양대학교 의과대학 소아과학교실) ;
  • 문수지 (한양대학교 의과대학 소아과학교실)
  • Received : 2005.11.04
  • Accepted : 2005.12.09
  • Published : 2006.04.15
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Abstract

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

목 적 : 신생아 루푸스는 전신성 홍반성 루푸스 산모의 자가 항체가 태반을 통해 태아에게로 넘어가 임상 증상을 보이는 질환으로 선천성 완전 방실 블록, 피부 병변, 간질환, 혈구 감소증을 특징으로 하는 질환이다. 이에 저자들은 전신성 홍반성 루푸스 산모의 출산아에서 선천성 완전 방실 블록의 빈도 및 출산아의 임상적 특징을 알아보고자 본 연구를 시행하였다. 방 법 : 1997년 1월부터 2005년 1월까지 한양대학교병원 류마티스 병원과 산부인과에서 진료받은 산모 55명의 57회의 임신결과와 태어난 49례의 신생아에 대한 임상기록을 후향적으로 검토하였다. 결 과 : 57례의 임신 중 5례(8.8%)의 자연유산, 1례(1.8%)의 사산이 있었고 15례(26.3%)의 조산, 8례(12.3%)의 부당 경량아의 빈도를 확인할 수 있었다. 57례의 임신 중 산전 초음파 검사에서 선천성 방실 블록 1례(1.8%)가 있었고 보호자 자의에 의해서 인공 유산이 행해졌다. 49례의 신생아 심전도 소견상 선천성 완전 방실 블록은 없었으며 모두 정상이었고 임상 기록상 피부병변이 있는 신생아는 없었다. 49례의 출산아 중 39례에서 혈액검사가 시행되었고 10례(25.6%)에서 혈액학적 이상이 있었다. 이 중 빈혈이 8례(20.5%), 중성구 감소증이 2례(5.1%), 혈소판 감소증이 3례(7.7%) 있었다. 자가 항체 anti-SSA(Ro)가 양성인 산모와 antiphospholipid 항체(aPL 항체)가 양성인 산모에서 미숙아 출산 빈도가 높았다(P=0.003, P=0.049). 항cardiolipin 항체(aCL 항체) 양성인 산모의 출산아가 인공환기요법을 받은 빈도가 높았다(P=0.018). 결 론 : 전신성 홍반성 루푸스 산모의 임신 중 선천성 완전 방실 블록의 빈도는 1.8%로 그 빈도가 낮았고 출산아 중 혈액학적 이상은 20% 이상에서 나타났다. 소홀히 하기 쉬운 피부병변에 대한 주의 깊은 추적 관찰로 신생아 홍반성 루푸스 진단에 관심을 가져야 할 것이다.

Keywords

References

  1. McCarty DJ, Manzi S, Medsger TA Jr, Ramsey-Goldman R, LaPorte RE, Kwoh CK. Incidence of systemic lupus erythematosus : race and gender differences. Arthritis Rheum 1995;38:1260-70 https://doi.org/10.1002/art.1780380914
  2. Buyon JP, Clancy RM. Neonatal lupus : Review of proposed pathogenesis and clinical data from the US based research registry for neonatal lupus. Autoimmunity 2003;36:41-50 https://doi.org/10.1080/0891693031000067340
  3. Brucato A, Buyon JP, Horsfall AC, Lee LA, Reichlin M. Fourth international workshop on neonatal lupus syndromes and the Ro/SSA-La/SSB System. Clin Exp Rheumatol 1999;17:130-6
  4. Cimaz R, Catelli L, Luzzana C, Panzeri P, Merni PL. Neonatal lupus syndromes. Isr Med Assoc J 2000;2:228-31
  5. Lee L. Neonatal lupus : clinical features, therapy, and pathogenesis. Curr Rheum Rep 2001;3:391-5 https://doi.org/10.1007/s11926-996-0009-3
  6. Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, et al. Autoimmune-associated congenital heart block : demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998;31:1658-66 https://doi.org/10.1016/S0735-1097(98)00161-2
  7. Waltuck J, Buyon J, Autoantibody-associated congenital heart block : outcome in mothers and children. Ann Intern Med 1994;120:544-51 https://doi.org/10.7326/0003-4819-120-7-199404010-00003
  8. Ellis FA, Bereston ES. Lupus erythematosus associated with pregnancy and menopause. AMA Arch Derm Syphilol 1952;65:170-6 https://doi.org/10.1001/archderm.1952.01530210049006
  9. Donaldson LB. De Alvarez RR. Further observations on lupus erythematosus associated with pregnancy. Am J Obstet Gynecol 1962;83:1461-73 https://doi.org/10.1016/S0002-9378(16)35991-9
  10. Nossent HC. Swaak TJ. Systemic lupus erythematosus. VI. Analysis of the interrelationship with pregnancy. J Rheumatol 1990;17:771-6
  11. Carmona F, Font J, Cervera R, Munoz F, Cararach V, Balasch J. Obstetrical outcome of pregnancy in patients with Systemic Lupus Erythematosus. A study of 60 cases. Eur J Obstet Gynecol Reprod Biol 1999;83:137-42 https://doi.org/10.1016/S0301-2115(98)00312-1
  12. Cimaz R, Spence DL, Hornberger L, Silverman ED. Incidence and spectrum of neonatal lupus erythematosus : a prospective study of infants born to mothers with anti-Ro autoantibodies. J Pediatr 2003;142:678-83 https://doi.org/10.1067/mpd.2003.233
  13. Brucato A, Frassi M, Franceschini F, Cimaz R, Faden D, Pisoni MP, et al. Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis : a prospective study of 100 women. Arthritis Rheum 2001;44:1832-5 https://doi.org/10.1002/1529-0131(200108)44:8<1832::AID-ART320>3.0.CO;2-C
  14. Dombroski RA. Autoimmune disease in pregnancy. Med Clin North Am 1989;73:605-21 https://doi.org/10.1016/S0025-7125(16)30660-5
  15. Kim WU, Min JK, Kwun NK, Park SH, Hong YS, Lee SH, et al. Obstetric outcomes in patients with systemic lupus erythematosus. J Korean Rheum Assoc 1997;4:131-8
  16. Shin JH, Lee EY, Lee CK, Cho YS, Kim A, Yoo B, et al. Fetal outcome and clinical feature during pregnancy in systemic lupus erythematosus. Korean J Med 2003;65:511-9
  17. Cunningham FG, MacDonald PC, Grant NF, Levono KJ. Williams Obstetrics. 19th ed. Norwalk, CT : Appleton & Lange, 1993:401-30
  18. Fernando A. Fetal growth retardation. Practical guide to high-risk pregnancy and delivery. 2nd ed. St. Louis : Mosby Tear Book, 1993:124-80
  19. National Center for Health Statistics. Advance report of final mortality statistics : 1989. Mon Vital Stat Rep 1992:40: 1-18
  20. Michaelsson M, Engle MA. Congenital complete heart block : an international study of the natural history. Cardiovasc Clin 1972;4:85-101
  21. Siren MK, Julkunen H, Kaaja R. The increased incidence of isolated congenital heart block in Finland. J Rhumatol 1998;25:1262-4
  22. Eronen M, Siren MK, Ekblad H, Tidanoja T, Julkunen H, Paavilainen T. Short and long term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 2000;106:1:86-91 https://doi.org/10.1542/peds.106.1.86
  23. Dubin A. Cardiac arrhythmias. In : Behrman RE, Klieman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 17th ed. Philadelphia : WB Sunders Co, 2004:1554-65
  24. Ramsey-Goldman R, Hom D, Deng JS, Ziegler GC, Kahl LE, Steen VD, et al. Anti-SS-A antibodies and fetal outcome in maternal systemic lupus erythematosus. Arthritis Rheum 1986;29:1269-73 https://doi.org/10.1002/art.1780291013
  25. Fu LS, Hawang B, Lee BH. Newborns of Chinese mother with systemic lupus erythematosus(SLE). Acta Paediatr Sin 1992;33:341-9
  26. Kim CR, Chang YP, Kim HS, Kim MJ, Kim BI, Choi JH, et al. Outcome in infants of mothers with systemic lupus erythematosus. J Korean Pediatr Soc 1993;36:791-804
  27. Kanagasegar S, Climz R, Kurien BT, Brucato A, Scofield RH. Neonatal lupus manifests as isolated neutropenia and mildly abnormal liver functions. J Rheumatol 2002;29:187-91
  28. Rader MD, O'Brien C, Liu YS, Harley JB, Reichlin M. Heterogeneity of the Ro/SSA antigen : different molecular forms in lymphocytes and red blood cells. J Clin Invest 1989;83:1293-8 https://doi.org/10.1172/JCI114014
  29. Lee LA, Reichlin M, Ruyle SZ, Weston WL. Neonatal lupus liver disease. Lupus 1993;5:333-8
  30. Lee LA, Sokol RJ, Buyon JP. Hepatobiliary disease in neonatal lupus : prevalence and clinical characteristics in cases enrolled in a national registry. Pediatrics 2002;109:E11 https://doi.org/10.1542/peds.109.1.e11
  31. McCune AB, Weston WL, Lee LA. Maternal and fetal outcome in neonatal lupus erythematosus. Ann Intern Med 1987;106:518-23 https://doi.org/10.7326/0003-4819-106-4-518