RT Journal Article
SR Electronic
T1 Health Outcomes of 215 Mothers of Children With Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1124
OP 1130
DO 10.3899/jrheum.210703
VO 49
IS 10
A1 Miniaoui, Imene
A1 Morel, Nathalie
A1 Lévesque, Kateri
A1 Maltret, Alice
A1 Driessen, Marine
A1 Masseau, Agathe
A1 Orquevaux, Pauline
A1 Piette, Jean-Charles
A1 Barriere, Francois
A1 Le Bidois, Jérome
A1 Georgin-Lavialle, Sophie
A1 Guettrot-Imbert, Gaëlle
A1 Le Guern, Véronique
A1 Mouthon, Luc
A1 Jallouli, Moez
A1 Deligny, Christophe
A1 Hachulla, Eric
A1 Romefort, Bénédicte
A1 ,
A1 Bonnet, Damien
A1 Costedoat-Chalumeau, Nathalie
YR 2022
UL http://www.jrheum.org/content/49/10/1124.abstract
AB Objective Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).Methods This retrospective study included anti-SSA/SSB antibody–positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.Results We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (P < 0.001), dry syndrome (P = 0.01), and parotid swelling (P = 0.05).Conclusion One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required.