Significance of Autoantibodies in Neonatal Lupus Erythematosus

Abstract

Autoantibodies produced by the mother and transported into the fetal circulation are of significant importance in the diagnosis of neonatal lupus syndromes. These humoral autoimmune findings provide an unique opportunity to assess the pathogenic role of autoantibodies against the Ro(SS-A)/La(SS-B) complex, most notably for congenital heart block. Current knowledge about the involved autoantibody-autoantigen systems, including recent therapeutic concepts of these autoimmune syndromes, is summarized.

Cloning and characterization of two human Ro52-specific monoclonal autoantibodies directed towards a domain associated with congenital heart block

“…This includes deposits of autoantibodies and complement components, as well as lymphocytic infiltrates found around the conduction system in the fetal heart tissue, emphasizing the importance of the immune system in mediating tissue destruction. Autoantibodies to Ro and La are detected in the mothers and affected children [1,6]. More specifically, antibodies to the Ro52 antigen show the highest correlation with heart block development [7,8].…”

“…Congenital heart block is a transferred autoimmune disease that affects children of mothers with autoantibodies to the intracellular Ro/SSA and La/SSB antigens (reviewed in Dö rner et al [1]). The Ro and La antibodies are associated with the rheumatic diseases Sjö gren's syndrome and systemic lupus erythematosus, but may also be found in asymptomatic individuals.…”

Central nervous system manifestations of neonatal lupus: a systematic review

“…5 Other common characteristic manifestations included transient dermatitis, as well as hepatobiliary and hematologic abnormalities which are also often reversible. Central nervous system (CNS) involvements, estimated in 1.4% of patients with NL, 6 are considered potential manifestations of NL and may result in long-term neurological complications in some cases. 7,8 Methods…”

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

“…60 kD Ro (SSA) und 48 kD La (SSB), die bei Patientinnen mit primärem Sjögren-Syndrom oder systemischem Lupus erythematodes auftreten, können transplazentar auf den Feten übertragen werden und durch Bindung an Ro-und La-Antigene im fetalen Erregungsleitungssystem eine Entzündungsreaktion mit anschlieûender Fibrosierung und Verkalkung auslösen [1,2,3]. Als Folge kann ein kongenitaler AV-Block mit schweren Bradykardien entstehen, der in 30 % letal verläuft [4,5] und bei zwei Drittel der überlebenden Kinder eine lebenslange Schrittmachertherapie erfordert [4].…”