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Aktuelle Rheumatologie 2003; 28(2): 102-107
DOI: 10.1055/s-2003-39384
Originalarbeit
© Georg Thieme Verlag Stuttgart · New York

Neonataler Lupus erythematodes und kompletter kongenitaler Herzblock

Neonatal Lupus Erythematosus and Complete Congenital Heart BlockE. Feist1 , R. Chaoui2 , B. Göldner3 , F. Hiepe4 , T. Dörner1
  • 1Klinik für Rheumatologie und Klinische Immunologie, Charité, Humboldt Universität Berlin
  • 2Klinik für Gynäkologie und Geburtshilfe, Charité, Humboldt Universität Berlin
  • 3Klinik für Pädiatrie, Charité, Humboldt Universität Berlin
  • 4Deutsches Rheumaforschungszentrum Berlin
Diese Studie wurde unterstützt vom Kompetenznetz Rheuma (Teilprojekt C3.5).
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Publication Date:
26 May 2003 (online)

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Zusammenfassung

Der neonatale Lupus erythematodes (NLE) umfasst vorrangig den kutanen NLE und den angeborenen AV-Block und wird durch eine antikörpervermittelte Entzündungsreaktion ausgelöst, wobei transplazentar übertragenen mütterlichen Autoantikörpern gegen den RNA-Proteinkomplex Ro(SS-A)/La(SS-B) eine pathogenetische Bedeutung zukommt. Die Krankheitsmanifestationen des NLE sind mit Ausnahme des kompletten kongenitalen Herzblockes (CCHB) durch den Abbau der mütterlichen Antikörper in der Zirkulation des Neugeborenen limitiert. Dabei gilt der CCHB als eine vital bedrohliche Komplikation, die insbesondere bei Müttern mit subklinischem Sjögren-Syndrom und koinzidenten Autoantikörpern gegen Ro(SS-A) und La(SS-B) auftreten kann. Die Arbeit gibt einen aktuellen Überblick zu diagnostischen und differenzialtherapeutischen Aspekten des angeborenen atrioventrikulären (AV-)Blockes, der enger mit einem mütterlichen Sjögren-Syndrom als mit einem systemischen Lupus erythematodes (SLE) assoziiert ist.

Abstract

Neonatal lupus erythematosus (NLE) comprises predominantly cutaneous neonatal lupus erythematosus and congenital atrioventricular block and is triggered by an inflammatory reaction mediated by antibodies. In this regard, pathogenetic significance is ascribed to transplacentally transfered maternal auto-antibodies against the RNA protein complex Ro(SS-A) / La(SS-B). The manifestations of NLE are limited due to degradation of the maternal antibodies, except, however, the manifestations of complete congenital heart block (CCHB). It must be beared in mind that CCHB is rated a life-threatening complication that can occur especially in mothers suffering from sub-clinical Sjögren’s syndrome co-incidentally with auto-antibodies against Ro(SS-A) and La(SS-B). The following article is an update review of the diagnostic and differential therapeutic aspects of neonatal atrioventricular block that is more closely associated with a maternal Sjögren’s syndrome than with systemic lupus erythematosus.

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PD Dr. med. Thomas Dörner

Klinik für Rheumatologie und Klinische Immunologie, Charité, Humboldt Universität

Schumannstraße 20/21

10117 Berlin

Email: thomas.doerner@charite.de