Intrauterine Therapie und kindliche Entwicklung bei vier Schwangerschaften einer Mutter mit Sjögren-Syndrom und Anti-Ro-Autoantikörpern

Abstract

Autoantibodies against 52/60kD-Ro proteins, frequently present in patients with Sjoegren's Syndrome or systemic lupus erythematosus, are transmitted to the fetus during pregnancy. These autoantibodies can damage the cardiac conductive system of the fetus and cause a complete atrioventricular block, with a mortality of 30 %. We report the intrauterine therapy during four pregnancies of the same mother with high 52/60kD-Ro autoantibodies and the outcome of her infants. Our patient with primary Sjoegren's Syndrome suffered an early miscarriage during her first pregnancy. During the second pregnancy, a fetal atrioventricular block was observed at 23 weeks of gestation. Although subsequently dexamethasone therapy and daily plasmaphereses were started, a cesarean section was necessary at 26 weeks due to hydrops fetalis. The girl died from the atrioventricular block after two days. During the third and fourth pregnancies, dexamethasone therapy was begun already at 7 weeks, and regular plasmaphereses at 15 weeks. The children were delivered by cesarean section at 32 and 36 weeks because of growth retardation. Both had normal electrocardiograms after birth and after 2 and 4 years. In pregnant women with connective tissue diseases, monitoring of anti Ro-autoantibodies and fetal heart function is important. Intrauterine therapeutic options are dexamethasone therapy to suppress maternal and fetal inflammatory reactions and repeated plasmaphereses to reduce autoantibody levels. Postnatal follow up of the infants for atrioventricular block and rheumatic manifestations is necessary.

Congenital Complete Atrioventricular Heart Block in a Pregnant Woman with Sjögren Syndrome: Prenatal Care Follow-Up and the Challenge of Intrauterine Treatment

“…Complete atrioventricular block is characterized by a complete dissociation between atrial and ventricular activity, with ventricular frequency usually below 60 bpm, in which fetuses with ventricular HR < 55 bpm have a poorer prognosis 6 due to the presence of endocardial fibroelastosis and signs of fetal hydrops. 7 8 …”

“…Another study reported a 10% to 15% increase in fetal HR. 6 However, despite the increase in fetal HR, there are no studies demonstrating a benefit in terms of survival of these fetuses with this treatment approach. 12 …”

Prevention and Treatment In Utero of Autoimmune-Associated Congenital Heart Block

“…Several have not identified any benefit in treating previously developed heart block with plasmapheresis. 37–40 Ruffatti et al described two cases with 2 nd degree heart block treated with a combination of plasmapheresis, dexamethasone and IVIG which improved to normal atrioventricular conduction, and eventual first degree heart block at the time of birth. 41 It is impossible to know if one particular therapy or the combination was responsible for these findings, however.…”