Kateri Levesque1, Nathalie Morel1, Alice Maltret2, Gabriel Baron3, Agathe Masseau4, Pauline Orquevaux5, Jean-Charles Piette6, Francois Barriere7, Jérome Le Bidois2, Laurent Fermont2, Olivier Fain8, Arnaud Theulin9, Francois Sassolas10, Philippe Pezard11, Zahir Amoura6, Gaëlle Guettrot-Imbert1, Delphine Le Mercier12, Sophie Georgin-Lavialle13, Christophe Deligny14, Eric Hachulla15, Luc Mouthon16, Philippe Ravaud17, Elisabeth Villain2, Damien Bonnet18, Nathalie Costedoat-Chalumeau19. 1. AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. 2. APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France. 3. AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. 4. Service de Médecine Interne, CHU Nantes, France. 5. CHU de Reims, Centre de compétence maladies auto-immunes et systémiques rares, Reims, France. 6. UPMC; AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. 7. Réanimation Pédiatrique AP-HM, CHU Timone Enfants, Marseille, France. 8. UPMC; AP-HP, Hôpital Saint Antoine, Paris, France. 9. Rhumatologie, Centre de référence maladies auto-immunes et systémiques rares, Strasbourg, France. 10. Hôpital Louis Pradel, Lyon, France. 11. CHU, Angers, France. 12. AP-HP, Gynécologie, Hôpital Necker, Paris, France. 13. UPMC; Hôpital Tenon, Paris, France. 14. CHU Fort de France, Martinique, France. 15. Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Université de Lille 2, Lille, France. 16. AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. 17. AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Department of Epidemiology, Mailman School of Public Health, Columbia University, NY, USA; French Cochrane Centre, Paris. 18. APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. 19. AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. Electronic address: nathalie.costedoat@gmail.com.
Abstract
BACKGROUND: Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB. METHODS: Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies. RESULTS: 214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p<0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p<0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB. CONCLUSION: In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.
BACKGROUND:Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB. METHODS: Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies. RESULTS: 214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p<0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with childdeath were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p<0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB. CONCLUSION: In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.
Authors: Megan E B Clowse; Amanda M Eudy; Elizabeth Kiernan; Matthew R Williams; Bonnie Bermas; Eliza Chakravarty; Lisa R Sammaritano; Christina D Chambers; Jill Buyon Journal: Rheumatology (Oxford) Date: 2018-07-01 Impact factor: 7.580
Authors: Julie Barsalou; Nathalie Costedoat-Chalumeau; Adey Berhanu; Cesar Fors-Nieves; Ummara Shah; Patrick Brown; Carl A Laskin; Nathalie Morel; Kateri Levesque; Jill P Buyon; Earl D Silverman; Peter M Izmirly Journal: Ann Rheum Dis Date: 2018-10-08 Impact factor: 19.103
Authors: Hieronymus T W Smeele; Marco W J Schreurs; Nathalie Costedoat-Chalumeau; Jerome M J Cornette; Radboud J E M Dolhain Journal: RMD Open Date: 2021-07