Tom 5, Nr 1 (2020)
Wytyczne / stanowisko ekspertów
Opublikowany online: 2020-05-11

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Eksport do Mediów Społecznościowych

Eksport do Mediów Społecznościowych

Toczeń rumieniowaty noworodków. Stanowisko Zespołu Ekspertów w sprawie diagnostyki, monitorowania oraz leczenia matki i noworodka

Bożena Kociszewska-Najman1, Magdalena Jaskólska1, Joanna Schreiber-Zamora1, Natalia Mazanowska2, Bronisława Pietrzak2, Bożena Werner3, Radosław Pietrzak3, Małgorzata Pańczyk-Tomaszewska4, Anna Woźniacka5, Mirosław Wielgoś2, Lidia Rudnicka6
Ginekologia i Perinatologia Praktyczna 2020;5(1):33-43.

Streszczenie

Poniższy artykuł przedstawia stanowisko Zespołu Ekspertów dotyczące postępowania w przypadku tocznia rumieniowatego noworodków (NLE, Neonatal Lupus Erythematosus). Jest to niezwykle rzadko występujące schorzenie i jednocześnie niezwykle rzadko opisywane w literaturze. Praca ma na celu uporządkowanie danych i rekomendację postępowania z noworodkiem. Mamy nadzieję, że znajdzie to odzwierciedlenie w codziennej praktyce zarówno położników i neonatologów, jak również pediatrów. NLE występuje z częstością 1 na 20 000 ciąż. Najczęściej spowodowany jest biernym przejściem przez łożysko autoprzeciwciał przeciwko Ro/SSA lub La/SSB. NLE może wystąpić u noworodków matek chorujących na toczeń układowy lub w przypadku występowania innych chorób związanych z obecnością przeciwciał wywołujących NLE, w tym toczeń podostry skórny (SCLE, Subacute Cutaneous Lupus Erythematosus), ogniskowy toczeń rumieniowaty (DLE, Discoid Lupus Erythematosus), zespół Sjögrena, mieszaną chorobę tkanki łącznej lub reumatoidalne zapalenie stawów. Objawami NLE mogą być: wrodzony blok serca (CHB, Congenital Heart Block), skórna postać NLE, zaburzenia hematologiczne czy zwiększenie aktywności enzymów wątrobowych. Najgroźniejszą postacią NLE jest całkowity blok przedsionkowo-komorowy występujący z częstością 1:15000 do 1:22000 żywych urodzeń. Najczęstszym objawem jest bradykardia płodu, która może być wykryta przypadkowo przy rutynowym osłuchiwaniu czynności serca płodu lub w trakcie badania ultrasonograficznego. Postać sercowa tocznia jest odpowiedzialna za 85–95% przypadków wrodzonego całkowitego bloku przedsionkowo-komorowego u noworodków z prawidłowym strukturalnie sercem. U kobiet z grupy ryzyka wystąpienia tocznia rumieniowatego noworodkowego konieczne jest wykonanie badań przesiewowych w kierunku obecności przeciwciał anty-Ro/SSA i anty-La/SSB, najlepiej jeszcze w okresie przedkoncepcyjnym. Zaleca się zwiększony nadzór położniczy w ośrodku dysponującym możliwością wykonania oceny echokardiograficznej (ECHO) płodu.

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Referencje

  1. Vanoni F, Lava SAG, Fossali EF, et al. Neonatal Systemic Lupus Erythematosus Syndrome: a Comprehensive Review. Clin Rev Allergy Immunol. 2017; 53(3): 469–476.
  2. Teixeira AR, Rodrigues M, Guimarães H, et al. Neonatal lupus - case series of a tertiary hospital. Acta Reumatol Port. 2017; 42(4): 318–323.
  3. Inzinger M, Salmhofer W, Binder B. Neonatal lupus erythematosus and its clinical variability. JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 2012; 10(6): 407–410.
  4. Morel N, Georgin-Lavialle S, Levesque K, et al. [Neonatal lupus syndrome: Literature review]. Rev Med Interne. 2015; 36(3): 159–166.
  5. Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002; 23(3): 279–299.
  6. Boros CA, Spence D, Blaser S, et al. Hydrocephalus and macrocephaly: new manifestations of neonatal lupus erythematosus. Arthritis Rheum. 2007; 57(2): 261–266.
  7. Brito-Zerón P, Izmirly PM, Ramos-Casals M, et al. The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol. 2015; 11(5): 301–312.
  8. Clowse MEB, Jamison M, Myers E, et al. A national study of the complications of lupus in pregnancy. Am J Obstet Gynecol. 2008; 199(2): 1–6.
  9. Yasmeen S, Wilkins EE, Field NT, et al. Pregnancy outcomes in women with systemic lupus erythematosus. J Matern Fetal Med. 2001; 10(2): 91–96.
  10. Buyon JP, Kim MY, Guerra MM, et al. Predictors of Pregnancy Outcomes in Patients With Lupus: A Cohort Study. Ann Intern Med. 2015; 163(3): 153–163.
  11. Clowse MEB, Magder LS, Witter F, et al. The impact of increased lupus activity on obstetric outcomes. Arthritis Rheum. 2005; 52(2): 514–521.
  12. Nalli C, Iodice A, Andreoli L, et al. Children born to SLE and APS mothers. Lupus. 2014; 23(12): 1246–1248.
  13. Neiman AR, Lee LA, Weston WL, et al. Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry. J Pediatr. 2000; 137(5): 674–680.
  14. Reed JH, Clancy RM, Lee KH, et al. Umbilical cord blood levels of maternal antibodies reactive with p200 and full-length Ro 52 in the assessment of risk for cardiac manifestations of neonatal lupus. Arthritis Care Res (Hoboken). 2012; 64(9): 1373–1381.
  15. Buyon JP, Kim MY, Copel JA, et al. Anti-Ro/SSA antibodies and congenital heart block: necessary but not sufficient. Arthritis Rheum. 2001; 44(8): 1723–1727.
  16. Cimaz R, Duquesne A. [Neonatal lupus syndromes]. Arch Pediatr. 2006; 13(5): 473–478.
  17. Bergman G, Eliasson H, Mohlkert LA, et al. Progression to first-degree heart block in preschool children exposed in utero to maternal anti-SSA/Ro52 autoantibodies. Acta Paediatrica. 2012; 101(5): 488–493.
  18. Pruetz JD, Miller JC, Loeb GE, et al. Prenatal diagnosis and management of congenital complete heart block. Birth Defects Res. 2019; 111(8): 380–388.
  19. Llanos C, Friedman DM, Saxena A, et al. Recurrence rates of cardiac manifestations associated with neonatal lupus and maternal/fetal risk factors. Arthritis Rheum. 2009; 60(10): 3091–3097.
  20. Cuneo BF, Strasburger JF, Niksch A, et al. An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease. J Matern Fetal Neonatal Med. 2009; 22(3): 233–238.
  21. Moak JP, Barron KS, Hougen TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol. 2001; 37(1): 238–242.
  22. Buyon JP, Hiebert R, Copel J, 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(7): 1658–1666.
  23. Falcini F, De Simone L, Donzelli G, et al. Congenital conduction defects in children born to asymptomatic mothers with anti-SSA/SSB antibodies: report of two cases. Ann Ital Med Int. 1998; 13(3): 169–172.
  24. Houssiau FA, Lebacq EG. Neonatal lupus erythematosus with congenital heart block associated with maternal systemic lupus erythematosus. Clin Rheumatol. 1986; 5(4): 505–508.
  25. Mori AD, Bruneau BG. TBX5 mutations and congenital heart disease: Holt-Oram syndrome revealed. Curr Opin Cardiol. 2004; 19(3): 211–215.
  26. Baruteau AE, Fouchard S, Behaghel A, et al. Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study. Eur Heart J. 2012; 33(5): 622–629.
  27. Baruteau AE, Behaghel A, Fouchard S, et al. Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block. Circulation. 2012; 126(12): 1469–1477.
  28. Seki A, Ishikawa T, Daumy X, et al. Progressive Atrial Conduction Defects Associated With Bone Malformation Caused by a Connexin-45 Mutation. J Am Coll Cardiol. 2017; 70(3): 358–370.
  29. Abnormal Positions and Relationships of the Heart in Anderson's Pediatric Cardiology, 4th Edition Editor-in-Chiefs: Gil Wernovsky. Elsevier. ; 2019.
  30. Neonatal Lupus Erythematosus. Definitions. 2020.
  31. Weston WL, Morelli JG, Lee LA. The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus. J Am Acad Dermatol. 1999; 40(5 Pt 1): 675–681.
  32. Neiman AR, Lee LA, Weston WL, et al. Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry. J Pediatr. 2000; 137(5): 674–680.
  33. Heelan K, Watson R, Collins SM. Neonatal lupus syndrome associated with ribonucleoprotein antibodies. Pediatr Dermatol. 2013; 30(4): 416–423.
  34. Silverman E, Jaeggi E. Non-cardiac manifestations of neonatal lupus erythematosus. Scand J Immunol. 2010; 72(3): 223–225.
  35. 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(1): E11.
  36. Zuppa AA, Riccardi R, Frezza S, et al. Neonatal lupus: Follow-up in infants with anti-SSA/Ro antibodies and review of the literature. Autoimmun Rev. 2017; 16(4): 427–432.
  37. Askanase AD, Izmirly PM, Katholi M, et al. Frequency of neuro-psychiatric dysfunction in anti-SSA/SSB exposed children with and without neonatal lupus. Lupus. 2010; 19(3): 300–306.
  38. Skog A, Eliasson H, Tingström J, et al. Long-term growth of children with autoantibody-mediated congenital heart block. Acta Paediatrica. 2013; 102(7): 718–726.
  39. Skog A, Tingström J, Salomonsson S, et al. Neurodevelopment in children with and without congenital heart block born to anti-Ro/SSA-positive mothers. Acta Paediatr. 2013; 102(1): 40–46.
  40. Martin V, Lee LA, Askanase AD, et al. Long-term followup of children with neonatal lupus and their unaffected siblings. Arthritis Rheum. 2002; 46(9): 2377.
  41. Izmirly PM, Saxena A, Kim MY, 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. Circulation. 2011; 124(18): 1927–1935.
  42. Levesque K, Morel N, Maltret A, et al. “Lupus néonatal” group, Group of collaborators. Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome. Autoimmun Rev. 2015; 14(12): 1154–1160.
  43. McHenry MM. Factors influencing longevity in adults with congenital complete heart block. Am J Cardiol. 1972; 29(3): 416–421.
  44. Eliasson H, Sonesson SE, Salomonsson S, et al. Swedish Congenital Heart Block Study Group. Outcome in young patients with isolated complete atrioventricular block and permanent pacemaker treatment: A nationwide study of 127 patients. Heart Rhythm. 2015; 12(11): 2278–2284.
  45. Dewey RC, Capeless MA, Levy AM. Use of ambulatory electrocardiographic monitoring to identify high-risk patients with congenital complete heart block. N Engl J Med. 1987; 316(14): 835–839.
  46. Michaëlsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation. 1995; 92(3): 442.
  47. Andreoli L, Bertsias GK, Agmon-Levin N, et al. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis. 2017; 76(3): 476–485.
  48. Ambrosi A, Wahren-Herlenius M. Congenital heart block: evidence for a pathogenic role of maternal autoantibodies. Arthritis Res Ther. 2012; 14(2): 208.
  49. 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 Am Coll Cardiol. 2010; 55(24): 2778–2784.
  50. Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014; 129(21): 2183–2242.
  51. Friedman DM, Kim MY, Copel JA, et al. Prospective evaluation of fetuses with autoimmune-associated congenital heart block followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study. Am J Cardiol. 2009; 103(8): 1102–1106.
  52. Jaeggi ET, Silverman ED, Laskin C, et al. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol. 2011; 57(13): 1487–1492.
  53. Mevorach D, Elchalal U, Rein AJ. Prevention of complete heart block in children of mothers with anti-SSA/Ro and anti-SSB/La autoantibodies: detection and treatment of first-degree atrioventricular block. Curr Opin Rheumatol. 2009; 21(5): 478–482.
  54. Rein AJ, Mevorach D, Perles Z, et al. Early diagnosis and treatment of atrioventricular block in the fetus exposed to maternal anti-SSA/Ro-SSB/La antibodies: a prospective, observational, fetal kinetocardiogram-based study. Circulation. 2009; 119(14): 1867–1872.
  55. Eliasson H, Sonesson SE, Sharland G, et al. Fetal Working Group of the European Association of Pediatric Cardiology. Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation. 2011; 124(18): 1919–1926.
  56. Buyon JP, Clancy RM, Friedman DM. Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside. Nat Clin Pract Rheumatol. 2009; 5(3): 139–148.
  57. Izmirly PM, Saxena A, Sahl SK, et al. Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system. Ann Rheum Dis. 2016; 75(6): 1161–1165.
  58. Jaeggi ET, Fouron JC, Silverman ED, et al. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation. 2004; 110(12): 1542–1548.
  59. Friedman DM, Llanos C, Izmirly PM, et al. Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: Results of a multicenter, prospective, open-label clinical trial. Arthritis Rheum. 2010; 62(4): 1138–1146.
  60. Pisoni CN, Brucato A, Ruffatti A, et al. Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study. Arthritis Rheum. 2010; 62(4): 1147–1152.
  61. Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, et al. Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation. 2012; 126(1): 76–82.
  62. Barsalou J, Jaeggi E, Laskin CA, et al. Prenatal exposure to antimalarials decreases the risk of cardiac but not non-cardiac neonatal lupus: a single-centre cohort study. Rheumatology (Oxford). 2017; 56(9): 1552–1559.
  63. Izmirly PM, Kim MY, Llanos C, et al. Evaluation of the risk of anti-SSA/Ro-SSB/La antibody-associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine. Ann Rheum Dis. 2010; 69(10): 1827–1830.
  64. Tsocos G, Gordon C, Smolen J. A Companion of Rheumatology, „ Systemic Lupus Erythematosus”.
  65. Waltuck J, Buyon JP. Autoantibody-associated congenital heart block: outcome in mothers and children. Ann Intern Med. 1994; 120(7): 544–551.
  66. Jaeggi ET, Hamilton RM, Silverman ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution's experience of 30 years. J Am Coll Cardiol. 2002; 39(1): 130–137.
  67. Cimaz R, Spence DL, Hornberger L, et al. Incidence and spectrum of neonatal lupus erythematosus: a prospective study of infants born to mothers with anti-Ro autoantibodies. J Pediatr. 2003; 142(6): 678.