First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry).

Autor: Fredi M; Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Science, ASST Spedali Civili, University of Brescia, Brescia, Italy., Andreoli L; Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Science, ASST Spedali Civili, University of Brescia, Brescia, Italy., Bacco B; S.S.d.D.U. Immunologia, Allergologia, A.O. Ordine Maurziano di Torino, Torino, Italy., Bertero T; S.S.d.D.U. Immunologia, Allergologia, A.O. Ordine Maurziano di Torino, Torino, Italy., Bortoluzzi A; UO e Sezione di Reumatologia, Dipartimento di Scienze Mediche, Universita' degli Studi di Ferrara, Cona, Italy., Breda S; Struttura Complessa Medicina Interna, ASST Papa Giovanni XXIII, Bergamo, Italy., Cappa V; Unit of Biostatistics, Biomathematics, and Bioinformatics, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy., Ceccarelli F; UO Complessa Reumatologia, Policlinico Umberto I- University La Sapienza, Rome, Italy., Cimaz R; Anna Meyer Children's Hospital, University of Firenze, Firenze, Italy., De Vita S; Clinica di Reumatologia, Azienda Sanitaria Universitaria Integrata di Udine, Udine, Italy., Di Poi E; Clinica di Reumatologia, Azienda Sanitaria Universitaria Integrata di Udine, Udine, Italy., Elefante E; UO Reumatologia, Dipartimento di Medicina Clinica e Sperimentale, Universita' di Pisa, Pisa, Italy., Franceschini F; Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Science, ASST Spedali Civili, University of Brescia, Brescia, Italy., Gerosa M; Istituto Ortopedico Gaetano Pini, University of Milan, Milan, Italy., Govoni M; UO e Sezione di Reumatologia, Dipartimento di Scienze Mediche, Universita' degli Studi di Ferrara, Cona, Italy., Hoxha A; Unità di Reumatologia, Dipartimento di Medicina, Università di Padova, Padova, Italy., Lojacono A; Department of Obstetrics and Gynecology, ASST Spedali Civili and University, Brescia, Italy., Marozio L; Ginecologia e Ostetricia 1, Dipartimento di Scienze Chirurgiche, Università di Torino, Turin, Italy., Mathieu A; Cattedra e Struttura Complessa di Reumatologia, Universita' degli Studi e AOU di Cagliari, Cagliari, Italy., Meroni PL; Immunorheumatology Research Laboratory, Istituto Auxologico Italiano, Milan, Italy., Minniti A; UO Complessa Reumatologia, Policlinico Umberto I- University La Sapienza, Rome, Italy., Mosca M; UO Reumatologia, Dipartimento di Medicina Clinica e Sperimentale, Universita' di Pisa, Pisa, Italy., Muscarà M; UO Reumatologia, ASST Ospedale Niguarda, Milan, Italy., Padovan M; UO e Sezione di Reumatologia, Dipartimento di Scienze Mediche, Universita' degli Studi di Ferrara, Cona, Italy., Piga M; Cattedra e Struttura Complessa di Reumatologia, Universita' degli Studi e AOU di Cagliari, Cagliari, Italy., Priori R; UO Complessa Reumatologia, Policlinico Umberto I- University La Sapienza, Rome, Italy., Ramoni V; Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Padova, Italy., Ruffatti A; Unità di Reumatologia, Dipartimento di Medicina, Università di Padova, Padova, Italy., Tani C; UO Reumatologia, Dipartimento di Medicina Clinica e Sperimentale, Universita' di Pisa, Pisa, Italy., Tonello M; Unità di Reumatologia, Dipartimento di Medicina, Università di Padova, Padova, Italy., Trespidi L; Dipartimento per la Salute della Donna, Bambino e Neonato, Fondazione Ospedale Maggiore, Milan, Italy., Zatti S; Department of Obstetrics and Gynecology, ASST Spedali Civili and University, Brescia, Italy., Calza S; Unit of Biostatistics, Biomathematics, and Bioinformatics, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy., Tincani A; Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Science, ASST Spedali Civili, University of Brescia, Brescia, Italy., Brucato A; Struttura Complessa Medicina Interna, ASST Papa Giovanni XXIII, Bergamo, Italy.; Dipartimento di Scienze Biomediche e Cliniche 'Sacco', Università degli Studi di Milano, Milan, Italy.
Jazyk: angličtina
Zdroj: Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2019 Feb 28; Vol. 6, pp. 11. Date of Electronic Publication: 2019 Feb 28 (Print Publication: 2019).
DOI: 10.3389/fcvm.2019.00011
Abstrakt: Objective: Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge. Method: Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) in utero or neonatal period (up to 27 days after birth). Result: Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected in utero (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 in utero , five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion ( p = 0.005, OR > 100; CI 95% 2.88->100 and hydrops ( p = 0.003, OR = 14.09; CI 95% 2.01-122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins. Conclusion: This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.
Databáze: MEDLINE
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