Women with Sjögren’s are at increased risk for both maternal complications and adverse neonatal outcomes. Adverse neonatal outcomes may occur from the disease itself, maternal complications and pregnancy-related disease flares, and teratogenic risk from medications used to treat Sjögren’s. Infants of women with Sjögren’s are at increased risk of preterm birth, admission to the neonatal intensive care unit, severe neonatal morbidity, and perinatal death. Among the neonatal anomalies, congenital heart disorders are the most common and are a major cause for concern. Anti-Ro/SSA and/or anti-La/SSB autoantibodies are associated with congenital heart block (CHB) in infants and mediate damage to the atrioventricular node. CHB is estimated to occur in approximately 2% of infants born to women with anti-Ro/SSA and 3% of infants born to women with anti-La/SSB. The recurrence rate of CHB in a subsequent pregnancy, once a mother has had a child with fetal heart block, jumps to nearly 20%. Autoantibody-associated CHB carries a substantial morbidity and mortality. CHB mortality varies from 12% to 43% in the literature, and it increases when the disease is associated with endocardial fibroelastosis or cardiomyopathy.
When CHB is diagnosed, intrauterine therapy is possible to increase the atrioventricular conduction speed and improve fetal outcome. Maternal treatment with fluorinated steroids, dexamethasone and betamethasone, can reduce the antibody-mediated inflammatory damage of nodal tissue and potentially reverse fetal heart block. These mothers will continue to require steroids for the remainder of their pregnancy. However, complete third-degree heart block often will not respond to steroids, and they will be discontinued after a 6-month trial. These newborns most likely will need a pacemaker upon birth. Alternative/additional therapies have included plasmapheresis, intravenous immunoglobulins, and beta-sympathomimetics.
CHB is a manifestation of what is termed “neonatal lupus”, as the Sjögren’s autoantibodies anti-Ro/SSA and/or anti-La/SSB can cross the placenta from mother to baby and cause this condition in newborns in either Sjögren’s or lupus. Neonatal lupus can appear as a characteristic red rash and sometimes can also cause liver abnormalities or low blood cell counts. These manifestations are almost always transient. However, their appearance should lead the gynecologist to suspect Sjögren’s or lupus in the mother and should be investigated further.
*article courtesy of The Sjögren's Book, Fifth Edition, Edited by Daniel J. Wallace, MD
