PT  - JOURNAL ARTICLE
AU  - Miniaoui, Imene
AU  - Morel, Nathalie
AU  - Lévesque, Kateri
AU  - Maltret, Alice
AU  - Driessen, Marine
AU  - Masseau, Agathe
AU  - Orquevaux, Pauline
AU  - Piette, Jean-Charles
AU  - Barriere, Francois
AU  - Le Bidois, Jérome
AU  - Georgin-Lavialle, Sophie
AU  - Guettrot-Imbert, Gaëlle
AU  - Le Guern, Véronique
AU  - Mouthon, Luc
AU  - Jallouli, Moez
AU  - Deligny, Christophe
AU  - Hachulla, Eric
AU  - Romefort, Bénédicte
AU  - ,
AU  - Bonnet, Damien
AU  - Costedoat-Chalumeau, Nathalie
TI  - Health Outcomes of 215 Mothers of Children With Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry
AID  - 10.3899/jrheum.210703
DP  - 2022 Oct 01
TA  - The Journal of Rheumatology
PG  - 1124--1130
VI  - 49
IP  - 10
4099  - http://www.jrheum.org/content/49/10/1124.short
4100  - http://www.jrheum.org/content/49/10/1124.full
SO  - J Rheumatol2022 Oct 01; 49
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.