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Congenital heart block caused by maternal autoantibodies anti-SSA/Ro and anti-SSB/La


Authors: M. Podrazil;  J. Bartůňková;  A. Šedivá
Authors‘ workplace: Ústav imunologie UK 2. LF a FN Motol, Praha
Published in: Čes. Revmatol., 16, 2008, No. 4, p. 163-168.
Category: Overview Reports

Overview

Neonatal lupus syndrome represents a model of passively acquired autoimmunity caused by specific maternal autoantibodies anti-SSA/Ro a anti-SSB/La, which cross the placenta and are associated with the development of congenital heart block in the fetus and/or a transient rash or various liver and blood cell abnormalites in the newborn. Complete form of congenital heart block is a permanent condition that entails significant morbidity, with nearly all affected infants requiring pacemakers. Perinatal mortality may aprroach up to 20 %. An intensive search is therefore ongoing for the specific etiopathophysiology and for diagnostic markers to approach and treat this disease.

Key words:
congenital heart block, neonatal lupus syndrome, autoantibodies anti-SSA/Ro a anti-SSB/La


Sources

1. Friedman DM, Rupel A, Buyon JP. Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus. Curr Rheumatol Rep 2007 May; 9(2): 101–8.

2. Michaelsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 1972; 4: 85–101.

3. Waltuck J, Buyon JP. Autoantibody-associated congenital heart block: outcome in mothers and children. Ann Intern Med 1994; 120: 544–551.

4. 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 nterimmunoelectrophoresis: a prospective study of 100 women. Arthritis Rheum 2001; 44: 1832–1835.

5. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congenital heart block: mortality, morbidity, and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998; 31: 1658–66.

6. Buyon JP, Clancy RM. Dying right to live longer: positing apoptosis as a link between maternal autoantibodies and congenital heart block. Lupus. 2008; 17(2): 86–90.

7. Wolin SL, Cedervall T. The La protein. Annu Rev Biochem 2002; 71: 375–403.

8. Chen X, Wolin SL. The Ro 60 kDa autoantigen: insights into cellular function and role in autoimmunity. J Mol Med 2004; 82: 232–239.

9. Espinosa A, Zhou W, Ek M, Hedlund M, Brauner S, Popovic K, et al. The Sjögren’s syndrome-associated autoantigen Ro52 is an E3 ligase that regulates proliferation and cell death. J Immunol 2006; 176: 6277–6285.

10. Salomonsson S, Dörner T, Theander E, Bremme K, Larsson P, Wahren-Herlenius M. A serologic marker for fetal risk of congenital heart block. Arthritis Rheum 2002; 46:1233–1241.

11. Cooley HM, Keech CL, Melny BJ, et al. Monozygotic twins discordant for congenital complete heart block. Arthritis Rheum 1997; 40: 381–4.

12. Fritsch C, Hoebeke J, Dali H, Ricchiuti V, Isenberg DA, Meyer O, Muller S. 52-kDa Ro/SSA epitopes preferentially recognized by antibodies from mothers of children with neonatal lupus and congenital heart block. Arthritis Res Ther 2005; 8:R4.

13. Clancy RM, Buyon JP, Ikeda K, Nozawa K, Argyle DA, Friedman DM, et al. Maternal antibody responses to the 52-kd SSA/Ro p200 peptide and the development of fetal conduction defects. Arthritis Rheum 2005; 52:3079–3086.

14. Eftekhari P, Salle L, Lezoualc’h F, Mialet J, Gastineau M, Briand JP, et al. Anti- SSA/Ro52 autoantibodies blocking the cardiac 5-HT4 serotoninergic receptor could explain neonatal lupus congenital heart block. Eur J Immunol 2000; 30: 2782–2790.

15. Kamel R, Eftekhari P, Clancy R, Buyon JP, Hoebeke J. Autoantibodies against the serotoninergic 5-HT4 receptor and congenital heart block: a reassessment. J Autoimmun 2005; 25: 72–76.

16. Salomonsson S, Sonesson SE, Ottosson L, Muhallab S, Olsson T, Sunnerhagen M, et al. Ro/SSA autoantibodies directly bind cardiomyocytes, disturb calcium homeostasis, and mediate congenital heart block. J Exp Med 2005; 201: 11–17.

17. Buyon JP, Clancy RM. Autoantibody-associated congenital heart block: TGFbeta and the road to scar. Autoimmun Rev 2005; 4: 1–7.

18. Solomon DG, Rupel A, Buyon JP. Birth order, gender and recurrence rate in autoantibody-associated congenital heart block: implications for pathogenesis and family counseling. Lupus 2003; 12: 646–647.

19. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congenital heart block: mortality, morbidity, and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998; 31: 1658–66.

20. Julkunen H, Eronen M. Long-term outcome of mothers of children with isolated heart block in Finland. Arthritis Rheum 2001; 44: 647–652.

21. Izmirly PM, Rivera TL, Buyon JP. Neonatal lupus syndromes. Rheum Dis Clin North Am 2007 May; 33(2): 267-85, vi.

22. Skog A, Wahren-Herlenius M, Sundström B, Bremme K, Sonesson SE. Outcome and growth of infants fetally exposed to heart block-associated maternal anti-Ro52/SSA autoantibodies. Outcome and growth of infants fetally exposed to heart block-associated maternal anti-Ro52/SSA autoantibodies. Pediatrics 2008 Apr.; 121(4): e803–9.

23. Glickstein J, Buyon J, Kim M, Friedman D. PRIDE investigators. The fetal Doppler mechanical PR interval: a validation study. Fetal Diagn Ther 2004 Jan-Feb; 19(1): 31–4.

24. Buyon JP, Askanase AD, Kim MY, Copel JA, Friedman DM. Identifying an early marker for congenital heart block: when is a long PR interval too long? Comment on the article by Sonesson et al. Arthritis Rheum 2005 Apr; 52(4): 1341–2.

25. Friedman DM, Kim MY, Copel JA, Davis C, Phoon CK, Glickstein JS, et al. PRIDE investigators: Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation. 2008 Jan 29; 117(4): 485–93. Epub 2008 Jan 14.

26. Blanford AT, Murphy BE. In vitro metabolism of prednisolone, dexamethasone, betamethasone, and cortisol by the human placenta. Am J Obstet Gynecol 1977; Feb 1;127(3): 264–7.

27. Saleeb S, Copel J, Friedman D, et al. Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody-associated congenital heart block. Arthritis Rheum 1999; 42: 2335–45.

28. Jaeggi ET, Fouron J-C, Silverman ED, et al. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 2004; 110: 1542–8.

29. Breur JMPJ, Visser GHA, Kruizc AA, et al. Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature. Ultrasound Obstet Gynecol 2004; 24: 467–72.

30. Robinson BV, Ettedgui JA, Sherman FS. Use of terbutaline in the treatment of complete heart block in the fetus. Cardiol Young 2001; 11: 683–6.

31. Carpenter RJ Jr, Strasburger JF, Garson A Jr, et al. Fetal ventricular pacing for hydrops secondary to complete atrioventricular block. J Am Coll Cardiol 1986; 8: 1434–6.

32. Van der Leij JN, Visser GH, Bink-Boelkens MT, et al. Successful outcome of pregnancy after treatment of maternal anti-Ro (SSA) antibodies with immunosuppressive therapy and plasmapheresis. Prenat Diagn 1994; 14: 1003–7.

33. Wong JP, Kwek KY, Tan JY, et al. Fetal congenital complete heart block: prophylaxis with intravenous gammaglobulin and treatment with dexamethasone. AustNZ J Obstet Gynaecol 2001; 41: 339–41.

34. Branch DW, Porter TF, Paidas MJ, et al. Obstetric uses of intravenous immunoglobulin: successes, failures and promises. J Allergy Clin Immunol 2001; 108: S133–8.

35. Shinohara K, Miyagawa S, Fujita T, et al. Neonatal lupus erythematosus: results of maternal corticosteroid therapy. Obstet Gynecol 1999; 93: 952–7.

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