Sjögren syndrome as the main maternal disease in mothers with babies affected with Ro-associated congenital heart block (Spanish Registry REBACC-GEAS-SEMI)

ESPINOSA GERARD; LUIS SÁEZ COMET; LUCIO PALLARÉS; ALEIDA MARTÍNEZ ZAPICO; FRANCESCA MITJAVILA; LUIS CAMINAL11,; ALEJANDRA FLORES-CHAVEZ; BRITO ZERON PILAR; PILAR ROSICH; JOSÉ ANTONIO VARGAS; JOSÉ ANTONIO GONZÁLEZ NIETO; CARLES TOLOSA VILELLA; JOSÉ MARIO SABIO; CÉSAR MORCILLO; RAMOS CASALS MANUEL; RETAMOZO SOLEDAD; ÁNGEL ROBLES; OLGA CAPDEVILA; LUIS TRAPIELLA; MÓNICA RODRIGUEZ; MERCEDES PÉREZ-CONESA; JOAQUIM ORISTRELL; KOSTOV, BELCHIN

Washington, DC

Simposio; 14th International Symposium on Sjögren's Syndrome; 2018

Johns Hopkins University School of Medicine

Objective. To analyze both the already-diagnosed and the underlying maternalautoimmune diseases of mothers with pregnancies affected by autoimmunecongenital heart block (CHB) associated with maternal anti-Roantibodies.Methods. The REBACC Spanish Multicenter Registry was created inMarch 2014. It is integrated by 12 centers with substantial experience inthe management of systemic autoimmune diseases. Autoimmune CHB wasdefined as: a) CHB of any type (I, II or III), fetal endocardial fibroelastosis(EFE) and/or cardiomyopathy, b) cardiac block diagnosed in utero or in thefirst postpartum month, and c) mothers carrying anti-Ro52, Ro60 and/or Laautoantibodies.Results. On October 2017, the REBACC Registry included a total of 45anti-Ro+ mothers with 50 single pregnancies with CHB. Mean maternal ageat the time of first affected pregnancy with CHB was 32.97 years (range:22-44). All mothers were anti-Ro60 (+), 17/17 anti-Ro52 (+) and 32/44(73%) anti-La (+). The mean gestational age at diagnosis of CHB was 23weeks (range 16-37). Information about fetal outcomes was available in45 pregnancies: AV blocks were of type I in 2 pregnancies (4.5%), typeII in 15 (33%) and type III in 27 (60%); 1 had an isolated EFE (2.5%);therapies used included dexamethasone or betamethasone (n=25), intravenousimmunoglobulins (n=5), and plasma exchanges (n=3); 11 pregnancieswere interrupted due to bad fetal prognosis (24%) and 34 (76%) weresuccessfully carried to term, and pacemaker implantation was required in18/34 babies (53%). At diagnosis of the first affected pregnancy, 31 (69%)mothers did not have any autoimmune disease and the remaining 14 (31%)had Sjögren syndrome (n=7), SLE (n=5) and undifferentiated autoimmunedisease (n=2). At the last visit, of the 33 women who initially did not haveany autoimmune diagnosis (none/undifferentiated autoimmune disease), 22(67%) developed a systemic autoimmune disease (SS in 16, SLE in 5 andSS+ SLE in 1). Also, 1 mother previously diagnosed with SLE, was subsequentlydiagnosed with Sjögren syndrome.Conclusions. At the last visit, 23/45 (51%) mothers with affected babieswith Ro-associated congenital heart block have a diagnosis of Sjögrensyndrome (overwhelmingly primary); in contrast, 70% of mothers have noidentified systemic autoimmune disease at the first affected pregnancy, asanti-Ro antibodies can be detected several years before SS is diagnosed.Autoimmune CHB is one of the first early signs of primary SS in women ofchildbearing age.